Preauricular tags and pits in the newborn: the role of hearing tests

The aim of this study was to determine the role of audiometry in a group of newborn infants with preauricular tags or pits. During the 2 years of prospective study, 26 infants were born with preauricular tags or pits (5.7 per 1000 livebirths) and were assessed for hearing impairment by performing behavioural audiometry on day 3 of life and brainstem-evoked response audiometry at 4 months of age. Five infants had associated congenital anomalies (19%). The behavioural audiometry was abnormal in seven infants (27%). The evoked response audiometry was abnormal in 4/23 (17%) newborn infants with isolated tags or pits, and revealed both conductive and/or sensorineural hearing impairment. The incidence of hearing impairment with isolated preauricular tag/pit was significantly higher ( p < 0.001) than the incidence reported in preschool children. We conclude that hearing assessment is recommended in the routine evaluation of the newborn with isolated preauricular tags or pits. This policy may facilitate early diagnosis and treatment of hearing impairment in this population.     

听力障碍儿童17例听力检测结果分析

目的探讨完善的听力学评估在听力障碍(听障)儿童听力检测及康复中的重要性。方法对17例既往诊断为听障的儿童进行常规声导抗测试、行为测听、耳声发射等综合听力学评估。结果听障儿童17例中3例听力正常,14例感音神经性听力损失,其中仅1例配戴助听器。结论听障儿童的听力学诊断应建立在完善的听力学评估的基础上。对听障儿童应进行长期听力学随访,直到获得可靠的双耳行为听阈、作出明确的听力学诊断为止。     

A Case of Beckwith-Wiedemann Syndrome with Conductive Hearing Loss

ABSTRACT. A case of Beckwith-Wiedemann syndrome with conductive hearing loss due to fixation of the stapes is described. This is the second case described in the literature. However, some patients may not have been diagnosed. We suggest that hearing of patients with BWS should be tested at intervals from early childhood.     

A Case of Beckwith-Wiedemann Syndrome with Conductive Hearing Loss

ABSTRACT. A case of Beckwith-Wiedemann syndrome with conductive hearing loss due to fixation of the stapes is described. This is the second case described in the literature. However, some patients may not have been diagnosed. We suggest that hearing of patients with BWS should be tested at intervals from early childhood.     

Hearing in children after meningococcal meningitis

OBJECTIVE: To review the outcome of sensorineural hearing loss in children who have had confirmed meningococcal meningitis. METHODS: A retrospective audit of children admitted to the Starship Children's Hospital with a confirmed diagnosis of meningococcal meningitis during a 4-year period. RESULTS: Sixty-five children had confirmed meningococcal meningitis. Dexamethasone was administered according to recommended guidelines in 46 children (72%) while 12 children (19%) did not receive steroids at any stage. All children were appropriately referred for hearing assessment. Forty-nine children (75%) had their hearing tested and reliable sensorineural evaluation was obtained in all but one case. Thirty-four children (70%) were seen up to 6 weeks from discharge, 86% by 12 weeks. Sixteen cases (25%) did not attend for audiological assessment. Sensorineural hearing impairment was found in two children (4.2%). CONCLUSIONS: Children with meningococcal meningitis were reliably referred for audiological assessment but 39% either failed to attend for an outpatient hearing evaluation (25%) or had an unacceptable delay between discharge and testing (14%). Of those reliably tested, two children (4.2%) had significant sensorineural hearing loss.     

Various aspects of hearing loss in newborns: A narrative review

Hearing loss is considered the most common birth defect. The estimated prevalence of moderate and severe hearing loss in a normal newborn is 0.1%-0.3%, while the prevalence is 2%-4% in newborns admitted to the newborn intensive care unit. Neonatal hearing loss can be congenital (syndromic or non-syndromic) or acquired such as ototoxicity. In addition, the types of hearing loss can be conductive, sensorineural, or mixed. Hearing is vital for the acquisition of language and learning. Therefore, early detection and prompt treatment are of utmost importance in preventing the unwanted sequel of hearing loss. The hearing screening program is mandatory in many nations, especially for high-risk newborns. An automated auditory brainstem response test is used as a screening tool in newborns admitted to the newborn intensive care unit. Moreover, genetic testing and screening for cytomegalovirus in newborns are essential in identifying the cause of hearing loss, particularly, mild and delayed onset types of hearing loss. We aimed to update the knowledge on the various aspects of hearing loss in newborns with regard to the epidemiology, risk factors, causes, screening program, investigations, and different modalities of treatment.     

Hearing improvement in patients with Fabry disease treated with agalsidase alfa

Aim : To describe the nature and prevalence of hearing loss in Fabry disease, and its response to enzyme replacement therapy (ERT) with agalsidase alfa. Methods : Fifteen male patients with Fabry disease were enrolled in a randomized, double-blind study and received placebo ( n = 8) or ERT ( n = 7) with agalsidase alfa for 6 months. This was followed by an open-label extension of 36 months thus far. Alongside this trial, an additional eight men and two women have so far received open-label ERT for between 6 and 30 months. Pure-tone audiometry, impedance audio-metry and otoacoustic emission testing were performed at 0 (baseline), 6, 18, 30 and 42 months. Results : Nine patients (36%) had bilateral and ten (40%) had unilateral high-frequency sensorineural hearing loss (SNHL). Three (12%) had unilateral middle ear effusions with conductive losses persisting beyond 6 months. Only five patients (20%) had normal hearing. The high-frequency SNHL deteriorated over the first 6 months in both placebo and active treatment groups by a median 6.3 dB ( p < 0.0001, Wilcoxon matched-pairs). This hearing loss subsequently improved above baseline by 1.5 dB at 18 months (p = 0.07), by 5.0 dB at 30 months ( p = 0.006) and by 4.0 dB at 42 months ( p = 0.01).
Conclusion : Significant hearing loss, usually high-frequency SNHL, is a common manifestation of Fabry disease in adults. α-Galactosidase A replacement therapy with agalsidase alfa appears to reverse the hearing deterioration in these patients. This improvement, however, is gradual, suggesting the need for long-term ERT.     

Infant,Maternal, and Hospital Factors' Role in Loss to Follow-up After Failed Newborn Hearing Screening

Objective

Completion of newborn hearing screening (NBHS) is recommended by 1 month old. Delays and loss to follow-up and documentation (LTF/LTD) after failed NBHS are common. Committees of experts have established hospital guidelines to reduce LTF/LTD. We aimed to identify maternal and infant factors associated with LTF/LTD and determine if adherence to hospital guidelines is associated with timely completion of follow-up screening.

Hearing loss in Turner syndrome

OBJECTIVE: To address the characteristics of hearing loss in patients with Turner syndrome (TS), we evaluated hearing levels of patients with TS and analyzed causative factors. STUDY DESIGN: Thirty-three patients with TS (8 to 40 years of age) were studied through the use of audiological measurements, and causative factors were explored. RESULTS: Twenty cases (35 of 66 ears tested) showed high-frequency (8 kHz) sensory neural hearing loss (HFQ-SNHL). Fifteen cases (26 ears) and 15 cases (24 ears) of the impaired 20 cases were unresponsive to distortion-product otoacoustic emissions and transient-evoked otoacoustic emissions, respectively. HFQ-SNHL showed little relation to the history of middle ear infection and puberty, although middle ear infections were seen in 11 of the 20 cases. The hearing thresholds at high frequencies were correlated with age and body height (P < .001). The age-dependent increase in hearing thresholds in the high frequencies was more apparent in patients with TS with monosomic 45, X than in those with the mosaic type (P < .05). CONCLUSIONS: More than 60% of patients with TS had HFQ-SNHL. Because the increase in hearing threshold at high frequencies was shown to depend on karyotype and aging, regular otological examination is important for the determination of proper treatment.     

Hearing loss in chronic myeloid leukemia

A 12-year-old girl presented with abdominal pain, fever, and hearing impairment of 6 months duration. She had massive hepatosplenomegaly and anemia. On the basis of her peripheral blood and bone marrow findings, she was diagnosed as chronic myeloid leukemia (CML) in chronic phase. Her hearing was assessed by brainstem evoked responses (BERA), which showed objective improvement in hearing with hydroxyurea. The rare occurrence of deafness in CML is reviewed and possible pathogenesis is discussed.     

高胆红素血症对新生儿听力的影响

目的研究高胆红素患儿听力障碍的发生率,探讨与听力障碍发生有关的高危因素。方法采用畸变产物耳声发射(DPOAE)对高胆红素血症患儿于病情稳定、出院前行听力筛查初筛,未通过者于生后42 d左右进行复筛,复筛未通过者行听觉脑干反应(ABR)确诊检查;选取产科出生正常新生儿作为对照。结果检测高胆红素血症患儿235例,初筛异常58例,占24.7%;复筛58例,异常11例,占18.9%;复筛未通过者3个月龄时行ABR检查,确诊听力障碍5例,高胆红素血症患儿听力障碍发生率为2.13%(5/235)。检测对照组正常新生儿182例,初筛异常18例,占9.9%;18例复筛均通过。结论高胆红素血症患儿是听力障碍发生高危人群。先天性巨细胞病毒感染、新生儿败血症及溶血病是导致发生听力障碍的危险因素。对高危新生儿应进行听力随访。     

HAEMOPHILUS INFLUENZAE MENINGITIS A Comparison between Chloramphenicol and Ampicillin Therapy with Special Reference to Impaired Hearing

ABSTRACT. 131 patients suffering from meningitis due to Haemophilus influenzae or parainfluenzae were re-examined after 1–15 years, using hospital records, questionnaires, and audiological examination, especially to compare chloramphenicol and ampicillin therapy. Mortality was 3.8%. Subdural effusions occurred in 14.5% of cases uni- or bilaterally. There was deafness in 2.3%, and moderate hearing loss in 8.4%. Convulsions appeared later in 6.9%. The final otucome was good in 60%. The most important factors in prognosis seemed to be the severity of the symptoms and the condition of the patient on admission to hospital. No clear difference was seen between the results of chloramphenicol and ampicillin therapy, but total loss of vestibular function was found in 3 cases in the ampicillin group, and in none in the chloramphenicol group. In mortality and deafness, the differences in outcome were similar, although not statistically significant. As these observations show, the therapy used in Haemophilus influenzae meningitis needs re-evaluation.     

Hearing loss after platinum treatment is irreversible in noncranial irradiated childhood cancer survivors

Cisplatin and carboplatin are effective antineoplastic agents. They are also considered to be potentially highly ototoxic. To date, no long-term follow-up data from well-documented cohorts with substantial numbers of childhood cancer survivors (CCS) with platinum-related hearing loss are available. Therefore, in this study, we studied the reversibility of ototoxicity from discontinuation of treatment onwards in a national cohort of platinum-treated survivors with hearing loss at the end of cancer treatment. Of the 168 CCS with follow-up audiograms, we longitudinally evaluated the course of hearing function in 61 CCS who showed hearing impairment at discontinuation of treatment according to the Münster criteria (>20 dB at ≥4–8 kHz). Survivors were treated with platinum (median total cumulative dose cisplatin: 480 mg/m² and median total cumulative dose carboplatin: 2520 mg/m²). Median follow-up time was 5.5 years (range: 1.0–28.8 years). The results showed that none of these survivors revealed improvement of hearing function even till 28.8 years after discontinuation of treatment (grade <2b during long-term follow-up). An increase in hearing loss with two or three Münster degrees was observed in five of 61 survivors after 1.6–19.6 years. Overall, this indicates that ototoxicity after platinum treatment may be irreversible and that longitudinal clinical audiological monitoring and care is required in long-term survivors of childhood cancer on a large scale.     

Hearing Impairment in Congenitally Hypothyroid Patients

Objective

Thyroid hormone is necessary for normal development of the auditory system. The aim of this study was to investigate the rate of hearing impairment in congenitally hypothyroid (CH) patients, and its relation with factors such as CH severity and age at starting treatment, during CH screening program in Isfahan.

Methods

Hearing acuity was assessed in two groups of children with (94 patients aged 4 months – 3 years) and without CH (450), between 2000-2006. Otoacostic emission (OAE) was performed by a two step method. After two tests without OAE signals bilaterally, they were referred for auditory brainstem response (ABR) test. Subjects with both OAE and ABR abnormal test results were considered to have hearing problem. Obtained data was compared in case and control group and also CH patients with and without hearing impairment.

Findings

Three (3.2%) of patients and 1 of control group (0.2%) were diagnosed with sensorineural hearing loss. The rate of hearing loss was not different significantly in two studied groups (P>0.05). There was no difference between age of starting treatment and first T4 and TSH level in CH patients with and without hearing loss (P>0.05). CH neonates with hearing impairment had thyroid dyshormonogenesis according to the follow up results.

Conclusion

The rate of hearing loss was low among our studied CH patients. It may be due to proper management of CH patients. In view of the fact that all CH neonates were dyshormonogentic and considering the relation between certain gene mutations and hearing impairment in CH patients, further studies with larger sample size, with regard to different etiologies of CH should be investigated to indicate the possible gene mutations related to hearing loss in CH.