Bilateral Sensorineural Hearing Impairment Research Articles

Identification of novel variants in BRF1 gene from patient with developmental delay, hearing abnormality, and nervous system anomalies.

Cerebellofaciodental syndrome characterized with dysmorphic features, intellectual disability, and brain anomalies. Now its clinical spectrum expanded more manifestations including bilateral sensorineural hearing impairment and inner ear malformation. Here, we report a 14-month-old boy with global developmental delay and hearing disorder. Whole exome sequencing (WES) revealed the compound heterozygous variants [NM_001519.4: c.652 T > G (p.W218G); c.915 + 1G > T] in the BRF1 gene which inherited from his parents, respectively. The MRI results showed hypoplastic cerebellar vermis, enlarged cisterna magna, and prominent fourth ventricle, the rehabilitation therapy failed to improve the symptoms for our patient. Our finding expands the genetic spectrum of BRF1 variants, which indicates patients with the developmental delay caused by BRF1 variants require other treatments instead of rehabilitation.

Revisiting Genetic Epidemiology with a Refined Targeted Gene Panel for Hereditary Hearing Impairment in the Taiwanese Population.

Hearing impairment is one of the most common sensory disorders in children, and targeted next-generation sequencing (NGS)-based genetic examinations can assist in its prognostication and management. In 2020, we developed a simplified 30-gene NGS panel from the original 214-gene NGS version based on Taiwanese genetic epidemiology data to increase the accessibility of NGS-based examinations. In this study, we evaluated the diagnostic performance of the 30-gene NGS panel and compared it with that of the original 214-gene NGS panel in patient subgroups with different clinical features. Data on the clinical features, genetic etiologies, audiological profiles, and outcomes were collected from 350 patients who underwent NGS-based genetic examinations for idiopathic bilateral sensorineural hearing impairment between 2020 and 2022. The overall diagnostic yield was 52%, with slight differences in genetic etiology between patients with different degrees of hearing impairment and ages of onset. No significant difference was found in the diagnostic yields between the two panels, regardless of clinical features, except for a lower detection rate of the 30-gene panel in the late-onset group. For patients with negative genetic results, where the causative variant is undetectable on current NGS-based methods, part of the negative results may be due to genes not covered by the panel or yet to be identified. In such cases, the hearing prognosis varies and may decline over time, necessitating appropriate follow-up and consultation. In conclusion, genetic etiologies can serve as references for refining targeted NGS panels with satisfactory diagnostic performance.

Analysis of molecular genetics and clinical characteristics of 3 children with Waardenburg syndrome

Objective:To analyze the molecular genetics and clinical characteristics of 3 children with syndromic deafness were analyzed to clarify their causative genes and genetic characteristics. Methods:The medical records of 3 children and their parents were collected and analyzed, including physical examination, hearing evaluation, temporal bone CT, and cranial MRI. Whole-exome sequencing（WES） was used to screen for pathogenic gene variants, and Sanger sequencing was used to verify the candidate positive variants in the probands and their parents. Results:All 3 patients were female with normal intelligence. Patient 1 and 3 had a family history of deafness, which conformed to the pattern of autosomal dominant inheritance. All three patients had bilateral profound sensorineural hearing impairment with bright-blue sclera. Other phenotypes included hypertelorism（patient 1）, multiple dyschromatosis（patient 2）, and yellowish hair（patient 2）, blepharoptosis（patient 3）. Patient 3 had bilateral vestibular enlargement, internal auditory canal enlargement, and bilateral inner ear malformations. Mother of patient 1 had only left mild hearing impairment; mother of patient 3 had bilateral hearing impairment with unilateral bright-blue sclera and yellowish hair. WES detected heterozygous variants, PAX3 c.811C>T, MITF c.632T>C, and SOX10 c.1359_1360 insGCCCCACA, in patient 1, 2, and 3, respectively. The variants in patient 1 and 3 were inherited from their mothers who had hearing impairment, and MITFvariant in patient 2 may be a spontaneous variation. The final diagnoses were that patient 1 with Waardenburg syndrome type 1（WS1）, and the mother of patient 1, patient 2, patient 3, and the mother of patient 3 with WS2. Conclusion:WS is a syndromic deafness, and the main clinical features include autosomal dominant inheritance and scleral pigment abnormalities. However, the findings of this study show that there is still phenotypic heterogeneity in WS even caused by the same gene variant, so it depends on genetic tests to confirm the diagnosis; The gene variant of patient 1 and 2 was never been reported in other patients, which expands the pathogenic variant spectrum of WS.

Deafness-Infertility Syndrome and Auditory Hallucinations

Deafness-Infertility Syndrome and Auditory Hallucinations

Outcome of language therapy in bimodal-fit children versus unilateral cochlear implant children in bilateral sensorineural hearing impairment: a case-control study

BackgroundTreating hearing-impaired children aims not only to improve their hearing but also to enhance language acquisition capability. In our community, the CI usually performed on one side because of financial issues at least for a period of time. Consequently, the brain may neglect the unfitted ear. Contralateral hearing aid is an alternative solution when bilateral CI is unavailable. Our purpose is to evaluate the language outcome in bimodal-fit children who using cochlear implant (CI) and contralateral hearing aid (HA) compared to children using unilateral cochlear implant only.ResultsIn this case-control study, 15 children who are using binaural-bimodal stimulation by unilateral CI and contralateral HA and 15 children using monaural cochlear implant received auditory training and language therapy. All participants have been assigned randomly from the Phoniatrics and Audiology clinics. Filtering of patients was made to get the two groups matched regarding age, sex, family motivation, age of implantation, and age of hearing impairment. Evaluation and language therapy were performed in the Phoniatrics clinic. Language progress in each group was compared over different time-points. Also, it was compared between the two groups in each time-point. Both groups revealed significant language improvement over time with intensive auditory training and language therapy. In addition, the bimodal-fit children showed better language and speech outcomes than the unilateral CI children in receptive semantics, expressive semantics, word class, mean length of utterance, and speech intelligibility. The differences were significant with P-values 0.047, 0.034, 0.03, 0.016, and 0.028, respectively, after 9 months of rehabilitation.ConclusionBimodal-fit children showed better improvement in language than the unilateral CI group. The contralateral hearing aid may be complementary to the unilateral cochlear implant by covering wider speech frequency range. Also, it prevents auditory deprivation and enables binaural hearing with positive impact on language outcome.

Effect of structured yoga program on functional balance, flexibility, and emotional status in students with bilateral sensorineural hearing impairment

Background: Children with bilateral sensorineural hearing impairment (SNHI) face various physiological, psychological, and sociological complications that require attention. The objective of the current study was to analyze the effect of a 1-month structured yoga program on functional balance, flexibility, and emotional status of the students with bilateral SNHI. Materials and Methods: The study was conducted as a controlled pre–post experimental method. Forty (n = 40) bilateral sensorineural hearing-impaired students (moderate–profound range) of ages 8–16 years were randomly divided into two equal groups, i.e., experimental and control. A one-hour structured yoga module was provided to the experimental group as an intervention for 30 days, while the control group followed a routine lifestyle. Outcome Measures: Static balance (SB) was assessed by single-leg stance test, dynamic balance (DB) by functional reach test, flexibility by sit and reach test, emotional status by Positive Affect and Negative Affect Scale (PANAS), and Self-Esteem recorded by Rosenberg Self-Esteem Scale. Results: The results revealed a significant improvement (P < 0.001) in physical parameters such as SB on the right leg, SB on the left leg, DB, and lumbar flexibility in the experimental group. Additionally, psychosocial parameters such as positive emotions, negative emotions, and self-esteem showed significant improvement in the experimental group. However, no significant change in any of the study parameters was observed in the control group (P > 0.05). Conclusion: These findings provide substantial evidence that a 1-month structured yoga program was an efficient method to improve the functional balance, flexibility, and emotional status in the students with bilateral SNHI.

Recurrent de novo WFS1 pathogenic variants in Chinese sporadic patients with nonsyndromic sensorineural hearing loss.

BackgroundHereditary hearing loss (HL) is heterogeneous in terms of their phenotypic features, modes of inheritance, and causative gene mutations. The contribution of genetic variants to sporadic HL remains largely expanding. Either recessive or de novo dominant variants could result in an apparently sporadic occurrence of HL. In an attempt to find such variants we recruited 128 Chinese patients with sporadic nonsyndromic sensorineural HL (NSHL) and performed targeted deafness multigene sequencing in these unrelated trios‐families to elucidate the molecular basis.MethodsWe analyzed a total of 384 available members (probands and their two parents) from 128 unrelated Chinese families presenting with bilateral sensorineural HL, in which previous screening had found no mutations with the GJB2, SLC26A4, and MT‐RNR1 genes. We used a targeted genomic enrichment platform to simultaneously capture exons, splicing sites, and immediate flanking intron sequences of 127 known deafness genes. Sanger sequencing was used to identify probands and their two parents segregating causative variants in the candidate gene.ResultsWe observed that two heterozygous de novo WFS1 mutations in exon 8: c.2051C>T (p.A684V) and c.2590G>A (p.E864K) in five families. The two de novo WFS1 mutations were found in 3.9% (5/128) of sporadic HL patients. We found that four of the five patients had the same de novo p.A684V mutation, and their audiograms showed symmetrical bilateral and profound sensorineural hearing impairments at all frequencies, but only the proband with de novo p.E864K mutation demonstrated significantly bilateral moderate low–mid frequency sensorineural HL. Our data suggest that this WFS1 p.A684V is likely to be a de novo mutational hot spot.ConclusionsWe found 3.9% (5/128) of sporadic NSHL is caused by de novo WFS1 mutations. Our data provide that the de novo p.E864K mutation is first identified and de novo p.A684V mutation is likely to be a mutational hot spot in WFS1. It is the first study to highlight that WFS1 gene with the two de novo mutations has been indicated to classify the distinct hearing impairment phenotypes. Furthermore, de novo p.A684V serves as a WFS1 mutational hot spot that was found in the Chinese population with sporadic childhood NSHL, and our study also provides pointers toward the necessity for sequencing of asymptomatic parents of a sporadic case with an apparent dominant pathogenic variant.

Perrault syndrome with neurological features in a compound heterozygote for two TWNK mutations: overlap of TWNK-related recessive disorders

BackgroundPerrault syndrome is a rare autosomal recessive disorder that is characterized by the association of sensorineural hearing impairment and ovarian dysgenesis in females, whereas males have only hearing impairment. In some cases, patients present with a diversity of neurological signs. To date, mutations in six genes are known to cause Perrault syndrome, but they do not explain all clinically-diagnosed cases. In addition, the number of reported cases and the spectra of mutations are still small to establish conclusive genotype–phenotype correlations.MethodsAffected siblings from family SH19, who presented with features that were suggestive of Perrault syndrome, were subjected to audiological, neurological and gynecological examination. The genetic study included genotyping and haplotype analysis for microsatellite markers close to the genes involved in Perrault syndrome, whole-exome sequencing, and Sanger sequencing of the coding region of the TWNK gene.ResultsThree siblings from family SH19 shared similar clinical features: childhood-onset bilateral sensorineural hearing impairment, which progressed to profound deafness in the second decade of life; neurological signs (spinocerebellar ataxia, polyneuropathy), with onset in the fourth decade of life in the two females and at age 20 years in the male; gonadal dysfunction with early cessation of menses in the two females. The genetic study revealed two compound heterozygous pathogenic mutations in the TWNK gene in the three affected subjects: c.85C>T (p.Arg29*), previously reported in a case of hepatocerebral syndrome; and a novel missense mutation, c.1886C>T (p.Ser629Phe). Mutations segregated in the family according to an autosomal recessive inheritance pattern.ConclusionsOur results further illustrate the utility of genetic testing as a tool to confirm a tentative clinical diagnosis of Perrault syndrome. Studies on genotype–phenotype correlation from the hitherto reported cases indicate that patients with Perrault syndrome caused by TWNK mutations will manifest neurological signs in adulthood. Molecular and clinical characterization of novel cases of recessive disorders caused by TWNK mutations is strongly needed to get further insight into the genotype–phenotype correlations of a phenotypic continuum encompassing Perrault syndrome, infantile-onset spinocerebellar ataxia, and hepatocerebral syndrome.

Representation of amplified speech at cortical level in good and poor hearing aid performers

IntroductionHearing aid users reject their own hearing aid because of annoyance with background noise. The reason for dissatisfaction is located anywhere from the hearing aid microphone to the integrity of neurons along the auditory pathway. In this preview, the output of hearing aid was recorded at the level of ear canal and at auditory cortex in good and poor hearing aid users, who were classified using acceptable noise level. ObjectiveTo study the representation of amplified speech in good and poor hearing aid performers. MethodsA total of 60 participants (age ranged 15–65 years) with moderate bilateral sensorineural hearing impairment grouped into good (n=35) and poor (n=25) hearing aid performers. Gap detection test and aided SNR 50 were administered. In addition, ear canal acoustic measures and cortical auditory evoked potentials were recorded in unaided and aided conditions at 65dB SPL. ResultsHearing aid minimally alters temporal contrast of speech reflected in envelope difference index. Although having similar temporal impairment, acoustic characteristics of amplified speech sounds and SNR 50 scores from both groups, the aided cortical auditory evoked potentials surprisingly showed significant earlier latencies and higher amplitudes in good performers than poor performers. In addition, good and poor performers classified based on annoyance level was predicted by latencies of 2N1 and 2P2 components of acoustic change complex. Further, a follow-up revealed hearing aid use has relation with acceptance towards noise. ConclusionParticipants who are willing to accept noise from those who are not willing to accept noise have subtle physiological changes evident at the auditory cortex, which supports the hearing aid usage.

A Comparative Study on the Development of Language Skills among the Children using Cochlear Implant and Hearing Aids

The purpose of this study was to document the performance of a group of children with moderately severe to severe hearing loss who use hearing aids on a range of language (speech-language, phonology and cognition) and to compare these results to children with severe to profound hearing loss, who have learned language through cochlear implants. This study involved 40 children with bilateral sensorineural hearing impairment, aged 6-18 years. Twenty children had moderately severe/severe hearing loss and used hearing aids, and 20 with severe to profound hearing loss and using cochlear implants. Communication and scholastic skills were assessed using speech recognition Tests and standardized measures of speech production, language, phonology, and cognition. In the present study, three variables: speech-language, phonology/literacy, and cognition were taken into consideration. This study suggests that there is an improvement in the development of language skills (speech-language, phonology & cognition) by using cochlear implant as an assistive device, when compared with hearing aids. Further research is required to evaluate the benefits of hearing aids and cochlear implants in children with hearing loss who are diagnosed and received intervention within the early years of life and to analyze the physiological, medical, and psychological effects on Scholastic performance in children with hearing impaired.

Phonological and grammatical production in children with developmental language disorder and children with hearing impairment

Children with hearing impairment (HI) are at an increased risk of developing speech and language problems similar to those of children with developmental language disorder (DLD), including difficulties with phonology and grammar. This study investigated similarities and differences in phonological and grammatical production between children with bilateral sensorineural HI ( n = 14) and children with DLD ( n = 30) between 4-6 years of age and age-matched controls with typical language development and normal hearing (TLD) ( n = 29), all with Swedish as their first language. Production of consonants, vowels, stress patterns and tonal word accents was assessed in a picture naming task, and in a word and nonword repetition task. Grammatical production was assessed for verb and noun morphology, and syntax. While performance for both children with HI and DLD were generally significantly below that of the controls with TLD, production of accents and syntax emerged as relative strengths. There were few differences between the between the children with HI and DLD, but noun–adjective agreement in predicative was more challenging for the children with HI. The results have implications for language assessment and planning of intervention.

Asymmetric sensorineural hearing impairment in the adult population

The relevance of the problem of a sensorineural hearing loss (ASNL) arises from the necessity of the special approaches to the diagnostics of this condition, the complications accompanying this pathology, and the difficulties encountered in the implementation of the methods designed for hearing rehabilitation of such patients. The objective of the present study was to estimate the prevalence of ASHL among the adult population. The sensorineural impairment of hearing was diagnosed in a total of 2456 (72%) examined patients presenting with hearing loss. To determine the presence of asymmetry of sensorineural hearing loss, we employed three counting techniques allowing (1) to calculate the difference between the average hearing thresholds at four frequencies within the range from 0.5 to 4 kHz (the difference was found to be 15 dB or more in 17% of the patients), (2) to calculate the difference between the degrees of hearing loss in the right and left ears (the difference was documented in 47% of the patients), (3) to calculate the difference between the hearing thresholds at least at a single frequency within the range from 0.125 to 8 kHz (he difference was found to be 15 dB or more in 71% of the patients). When using the third method to characterize asymmetry of hearing impairment, it was identified in most patients (53%) at one or two frequencies. Moreover, there was a large number of the patients (13%) with asymmetry apparent over the entire frequency range. A high degree of threshold asymmetry (in excess of 40 dB) was more often noted in the mid-frequency range. In the majority of the patients, the asymmetry manifested itself as the different degree of bilateral sensorineural hearing impairment (51%) or unilateral sensorineural hearing loss with the normal hearing ability preserved in the contralateral ear (35%). The results of the present study give evidence of the necessity of developing a reliable method for the identification of clinically significant ACNL.

Is Health Care Support Associated with Better Educational Achievement in Sensorineural Hearing Impaired Students with Overlooked Recurrent Otitis Media?

Background: Otitis media is an important health care problem in school-age children. Moreover, the prevalence of allergic rhinitis, a major and common cause of otitis media, is increasing among children. Recurrent otitis media may occur without any noticeable symptoms and affect the auditory threshold (AT). Only a few decibles increase in hearing loss does not have the same effect on the hearing ability of individuals with different degrees of hearing loss. With this background in mind, in this study, we aimed to determine whether diagnosis and medical treatment of overlooked recurrent otitis media are associated with the educational achievement of students with sensorineural hearing impairment. Methods: The present study with a pretest-posttest design was conducted on 30 students (aged 12 - 20 years) with bilateral sensorineural hearing impairment, AT above 30 dB HL in the better ear, and recurrent (2 episodes) middle ear infection over 9 months during 2012 - 2014. The participants were examined regularly and received medical treatment when indicated. The mean scores of total average, Persian literature (eg, reading, writing, and spelling), and mathematics were measured to assess the subjects’ educational achievement. Results: Based on the findings, the total average scores significantly changed from 16.2 to 17.2. Also, the students’ Persian literature (from 16.8 to 18) and mathematics (from 13.8 to 16.7) scores were significantly improved. In addition, AT considerably decreased after the medical intervention (from 73 ± 14.7 to 56.3 ± 13.8 dB HL). Conclusions: It seems that detection and treatment of overlooked middle ear infection in hearing impaired students, as part of comprehensive health care services, can improve their AT, diminish their hearing disabilities, and ameliorate their educational difficulties; consequently, provision of health care services is associated with better educational achievement.

Radioiodine Treatment For Hyperthyroidism In A Patient With Pendred Syndrome

ABSTRACT Objective: To report a case of Pendred syndrome accompanied by hyperthyroidism that was treated with radioiodine. Pendred syndrome is an autosomal recessive disorder characterized by bilateral sensorineural hearing impairment, goiter, and impaired iodide organification. The majority of patients with Pendred syndrome are euthyroid or hypothyroid, and hyperthyroidism is rare. Thus far, there are no reported cases of Pendred syndrome with 131I-treated hyperthyroidism. Methods: A 39-year-old woman with congenital hearing impairment visited our clinic because of easy fatigability and body weight loss. Results: She had a diffuse goiter (estimated thyroid weight, 65 g) and subclinical thyrotoxicosis. 99mTechnetium pertechnetate scintigraphy revealed diffuse thyroidal uptake, but thyroid autoantibodies were absent. Since the therapeutic response to a single 131I dose was poor, the patient underwent a second 131I dose (13 mCi each) for hyperthyroidism. Seven years after the second 131I dose, she was euthy...

Sensorineural Hearing Impairment and Subclinical Atherosclerosis in Rheumatoid Arthritis Patients Without Traditional Cardiovascular Risk Factors.

This study aims to evaluate the association of hearing impairment with carotid intima-media thickness and subclinical atherosclerosis in rheumatoid arthritis (RA) patients. A total of 41 RA patients (2 males, 39 females; mean age 46.5±10.2 years; range 20 to 63 years) with no known traditional cardiovascular risk factors were included. Routine clinical and laboratory assessments for RA patients were performed. Pure tone air (250-8000 Hz) and bone conduction (250-6000 Hz) thresholds were obtained, tympanograms and impedance audiometry were conducted. Sensorineural hearing impairment was defined if the average thresholds were ≥25 decibels. Carotid intima-media thickness was assessed and classified with a cut-off point of 0.6 mm. Thirteen patients (31.7%) had normal audition, while 28 (68.3%) had hearing impairment. Of these, 22 had bilateral sensorineural hearing impairment. Four patients had conductive hearing impairment (right in three patients and left in one patient). Patients with sensorineural hearing impairment had increased carotid intima-media thickness in the media segment of carotid common artery compared to patients with normal hearing (right ear p=0.007; left ear p=0.075). Thickening of the carotid intima-media thickness was associated with sensorineural hearing impairment in RA patients. Rheumatoid arthritis patients should be evaluated by carotid intima-media thickness as a possible contributing factor of hearing impairment in patients without cardiovascular risk factors.

Better-ear glimpsing in hearing-impaired listeners.

When competing speech sounds are spatially separated, listeners can make use of the ear with the better target-to-masker ratio. Recent studies showed that listeners with normal hearing are able to efficiently make use of this "better-ear," even when it alternates between left and right ears at different times in different frequency bands, which may contribute to the ability to listen in spatialized speech mixtures. In the present study, better-ear glimpsing in listeners with bilateral sensorineural hearing impairment, who perform poorly in spatialized speech mixtures, was investigated. The results suggest that this deficit is not related to better-ear glimpsing.

Two sporadic patients of Perrault syndrome with ovarian dysgenesis and sensorineural deafness

Perrault syndrome is an autosomal recessive disorder characterized by sensorineural deafness and ovarian dysgenesis. Some patients also have neurologic abnormalities, including cerebellar ataxia, nystagmus, polyneuropathy and mild mental retardation. The syndrome is known to be caused by mutations in HSD17B4 or HARS2 until now but few patients were reported. We report on two sporadic Korean patients of Perrault syndrome with ovarian dysgenesis and sensorineural deafness. Patient 1 : A 14 year-6 month female patient presented with delayed puberty. She had bilateral sensorineural hearing impairment. Her neurological findings were normal and She had no mental retardation. She had normal karyotype (46, XX) and hypergonadotropic hypogonadism. Pelvic ultrasound showed a small uterus and ovaries. Brain MRI was normal. Patient 2 : A 16 year-11 month female presented with delayed puberty and neurologic manifestations. She had ataxia, dyspraxia and weakness. She had no mental retardation and brain MRI was normal. She had multiple Cafe au lait spots and long slender fingers but no Marfanoid features. Laboratory tests revealed hypergonadotropic hypogonadism and normal female karyotype (46, XX). Pelvic ultrasound examination showed a hypoplastic uterus and small ovaries. Because Perrault syndrome has clinical variability and genetically heterogeneous, we have to inspect additional findings and neurological abnormalities. And further studies will be required to ascertain the common causative mutation of this syndrome.

Novel COCH mutation in a family with autosomal dominant late onset sensorineural hearing impairment and tinnitus

This report describes a three generation family with late onset bilateral sensorineural hearing impairment (BLSNHI) and tinnitus in which a novel mutation in the COCH gene was identified after a genome-wide linkage approach. The COCH gene is one of the few genes clinically examined when investigating the etiology of autosomal dominant late onset hearing impairment. Initially mutations in the COCH gene were only reported in exons 4 and 5, coding for the LCCL protein domain. More recently, additional mutations have been identified in exon 12, the only mutations identified outside of the LCCL domain. Currently clinical genetic testing for the COCH gene primarily focuses on identifying mutations in these three exons. In this study, we identify a novel mutation in the COCH gene in exon 11, which, like the exon 12 mutations, falls within the vWFA2 protein domain. This finding reinforces the need for clinical genetic screening of the COCH gene to be expanded beyond the current limited exon screening, as there is now more evidence to support that mutations in other areas of this gene are also causative of a similar form of late onset BLSNHI.

ブタ咬傷20日後に両側感音性難聴をともなうブタ連鎖球菌性髄膜炎を発症した1例

Streptococcus suis (S. suis) is a zoonotic pathogen in pigs, which can be transmitted to humans by close contact. Meningitis is the most common clinical manifestations of S. suis infection and hearing impairment is a frequent complication. The risk of S. suis meningitis is higher in people who work in the swine industry. The patient was a 53-year-old woman working in the swine industry, who developed headache and fever 20 days after a swine bite. She was diagnosed as meningitis and S. suis was detected in the cerebrospinal fluid. We treated her with ceftriaxone, vancomycin, and dexamethasone, and signs of meningeal irritation diminished three days after admission. However, bilateral sensorineural hearing impairment occurred on the ninth day after admission. We added methylprednisolone (500 mg, 2 days) but moderate hearing impairment remained on the left. Antibiotic therapy should be considered for wounds of people involved in the swine industry for preventing S. suis infection.When S. suis meningitis occurs, symptoms of hearing impairment must be monitored carefully.

1262 Prevalence of Hearing Impairments at Age Four Years in Extremely Preterm Infants

Background and AimExtremely preterm infants are at increased risk of permanent hearing loss. However, population-based data in infants born with less than 27 weeks gestation are scarce. The aim of...