Neurodevelopmental outcome in children with congenital cytomegalovirus infection: A prospective multicenter cohort study.

BACKGROUND: Congenital cytomegalovirus infection (cCMV) is the most common congenital infection worldwide and is a major cause of neurodevelopmental impairment in children. At this point there are insufficient data on neurodevelopmental outcome of children with cCMV, both symptomatic and asymptomatic. AIM: This study aimed to describe the neurodevelopmental outcome in a large prospective cohort of children with cCMV. METHODS: All children with cCMV, included in the Flemish cCMV register, were eligible for this study. Data on neurodevelopmental outcome was available in 753 children. Data on neuromotor, cognitive, behavioral, audiological and ophthalmological outcome were analyzed. RESULTS: Neurodevelopmental outcome was normal in 530/753 (70,4 %) at any age of last follow-up. Mild, moderate and severe neurodevelopmental impairment was found in 128/753 (16,9 %), 56/753 (7,4 %) and 39/753 (5,2 %), respectively. Adverse outcome is found both in the symptomatic and asymptomatic children (53,5 % versus 17,8 %). Autism spectrum disorder (ASD) was diagnosed more often than in the general population in Flanders (2,5 % versus 0,7 %). Speech and language impairment was found in 2 %, even in absence of hearing loss. CONCLUSION: Both symptomatic and asymptomatic cCMV children are at risk of sequelae, with higher risk in case of first trimester infection. During follow-up of this population, special attention should be given to the audiological follow-up, the presence of hypotonia at young age, the possible higher risk of ASD and the risk of speech and language impairment even in absence of hearing loss. Our results emphasize the need for multidisciplinary neurodevelopmental follow-up of all cCMV infected children.

Longitudinal analysis of the audiological phenotype in osteogenesis imperfecta: a follow-up study.

OBJECTIVE: This prospective study involved a longitudinal analysis of the progression of hearing thresholds in patients with osteogenesis imperfecta. METHODS: Audiometric results from 36 osteogenesis imperfecta patients (age range, 6-79 years) were compared between two test times with an average interval of 4 years. Audiometric evaluation included acoustic admittance measurements, acoustic stapedial reflex measurements, pure tone audiometry and otoacoustic emissions testing. RESULTS: Air conduction pure tone average, corrected for sex and age, and bone conduction pure tone average increased significantly in the study population (p < 0.05 and p < 0.001, respectively). In 14.3 per cent of the evaluated ears, an alteration in type and/or severity of hearing loss was observed. CONCLUSION: After an average time interval of four years, significant changes in hearing status occurred in a population of osteogenesis imperfecta patients. These findings highlight the importance of regular audiological follow up in osteogenesis imperfecta patients, including audiometry, and measurements of acoustic admittance, acoustic stapedial reflexes and otoacoustic emissions.

The relationship between tinnitus pitch and parameters of audiometry and distortion product otoacoustic emissions.

OBJECTIVES: Chronic tinnitus is associated with reduced auditory input, which results in changes in the central auditory system. This study aimed to examine the relationship between tinnitus pitch and parameters of audiometry and distortion product otoacoustic emissions. For audiometry, the parameters represented the edge frequency of hearing loss, the frequency of maximum hearing loss and the frequency range of hearing loss. For distortion product otoacoustic emissions, the parameters were the frequency of lowest distortion product otoacoustic emission amplitudes and the frequency range of reduced distortion product otoacoustic emissions. METHOD: Sixty-seven patients (45 males, 22 females) with subjective chronic tinnitus, aged 18 to 73 years, were included. RESULTS: No correlation was found between tinnitus pitch and parameters of audiometry and distortion product otoacoustic emissions. However, tinnitus pitch fell mostly within the frequency range of hearing loss. CONCLUSION: The current study seems to confirm the relationship between tinnitus pitch and the frequency range of hearing loss, thus supporting the homeostatic plasticity model.

The impact of tinnitus characteristics and associated variables on tinnitus-related handicap.

OBJECTIVE: This study aimed to determine the characteristics of tinnitus and tinnitus-related variables and explore their possible relationship with tinnitus-related handicap. METHODS: Eighty-one patients with chronic tinnitus were included. The study protocol measured hearing status, tinnitus pitch, loudness, maskability and loudness discomfort levels. All patients filled in the Tinnitus Sample Case History Questionnaire, the Hyperacusis Questionnaire and the Tinnitus Handicap Inventory. The relationship of each variable with the Tinnitus Handicap Inventory score was evaluated by univariate and multivariate analyses. RESULTS: Five univariables were associated with the Tinnitus Handicap Inventory score: loudness discomfort level, subjective tinnitus loudness, tinnitus awareness, noise intolerance and Hyperacusis Questionnaire score. Multiple regression analysis showed that the Hyperacusis Questionnaire score and tinnitus awareness were independently associated with the Tinnitus Handicap Inventory score. CONCLUSION: Hyperacusis and tinnitus awareness were independently associated with the Tinnitus Handicap Inventory score. Questionnaires on tinnitus and hyperacusis are especially suited to providing additional insight into tinnitus-related handicap and are therefore useful for evaluating tinnitus patients.

Hearing loss in Waardenburg syndrome: a systematic review.

Waardenburg syndrome (WS) is a rare genetic disorder characterized by hearing loss (HL) and pigment disturbances of hair, skin and iris. Classifications exist based on phenotype and genotype. The auditory phenotype is inconsistently reported among the different Waardenburg types and causal genes, urging the need for an up-to-date literature overview on this particular topic. We performed a systematic review in search for articles describing auditory features in WS patients along with the associated genotype. Prevalences of HL were calculated and correlated with the different types and genes of WS. Seventy-three articles were included, describing 417 individual patients. HL was found in 71.0% and was predominantly bilateral and sensorineural. Prevalence of HL among the different clinical types significantly differed (WS1: 52.3%, WS2: 91.6%, WS3: 57.1%, WS4: 83.5%). Mutations in SOX10 (96.5%), MITF (89.6%) and SNAI2 (100%) are more frequently associated with hearing impairment than other mutations. Of interest, the distinct disease-causing genes are able to better predict the auditory phenotype compared with different clinical types of WS. Consequently, it is important to confirm the clinical diagnosis of WS with molecular analysis in order to optimally inform patients about the risk of HL.

Middle ear damages.

Middle ear damages. The eardrum and middle ear are often exposed to blunt and penetrating trauma, blasts, thermal or caustic injuries. These injuries may result in tympanic membrane perforation, middle ear haemorrhage, dislocation and fracture of the ossicular chain, perilymphatic fistula and damage to the chorda tympani and/or facial nerve. In case of life-threatening injuries and/or mass casualty incidents, middle ear trauma obviously does not take highest priority. However, middle ear lesions should be suspected and recognized as early as possible. After meticulous history taking, physical examination consists of cranial nerve evaluation, thorough inspection of the outer ear, otoscopy and assessment of hearing and vestibular function. In the majority of cases, traumatic tympanic membrane perforations by penetrating and blunt injuries have a good prognosis with spontaneous resolution. Tympanic membrane perforations from blast trauma, thermal or caustic injuries are less likely to heal spontaneously. Perforations lasting six months after injury warrant surgery. A high resolution CT scan of the temporal bone is required in case of immediate complete facial nerve paralysis and when oval window pathology or perilymphatic fistula is suspected. Early surgical intervention is needed in case of early onset facial nerve paralysis, when there is suspicion of a perilymphatic fistula with persisting or increasing vestibular symptoms or neurosensory hearing loss and in case of vestibular dislocation of the stapes footplate. When ossicular chain damage is suspected, elective tympanoplasty is indicated. As any traumatic tympanic membrane perforation runs the risk of cholesteatoma formation, biannual follow-up during a minimum of two years is recommended.

Olivocochlear efferent reflex strength in vestibular schwannoma patients.

PROBLEM/OBJECTIVE: The olivocochlear reflex strength can be measured using contralateral acoustic stimulation (CAS) of transient evoked otoacoustic emissions (TEAOEs). The efferent suppression (ES) thus reflects the functional intactness of the efferent auditory system. In patients with unilateral vestibular schwannoma (VS), the results of ES are unclear. METHODS: This cross-sectional study evaluated click and noise intensity conditions for measuring ES using CAS of TEOAEs in nine patients with unilateral VS and in gender-, age- and ear side-matched control subjects. RESULTS: Two optimal click and noise intensity level combinations were identified. This pilot study compared the amount of ES between tumour and non-tumour ears versus control ears in patients with unilateral VS, but there were no statistically significant differences. CONCLUSION: Future studies should include a larger population of unilateral VS patients with different grades of hearing loss.

Nasalance and nasality in children with cochlear implants and children with hearing aids.

OBJECTIVES: In prelingually deaf children, many speech production aspects including resonance, are known to be problematic. This study aimed to investigate nasality and nasalance in two groups of prelingually hearing impaired children, namely deaf children with a cochlear implant (CI) and moderate-to-severely hearing impaired hearing aid (HA) users. The results of both groups are compared with the results of normal hearing children. Besides, the impact of the degree of hearing loss was determined. METHODOLOGY: 36 CI children (mean age: 9;0y), 25 HA children (mean age: 9;1y) and 26 NH children (mean age: 9;3y) were assessed using objective assessment techniques and perceptual evaluations in order to investigate the nasal resonance of the three groups. Ten HA children had thresholds above 70dB (range: 91dB-105dB) and fifteen below 70dB (range: 58dB-68dB). The Nasometer was used for registration of the nasalance values and nasality was perceptually evaluated by two experienced speech therapists using a nominal rating scale (consensus evaluation). RESULTS: For nasal stimuli, both CI children and HA children showed lower nasalance values in comparison with NH children. The opposite was observed for the oral stimuli. In both hearing impaired groups, cul-de-sac-resonance was observed on a significantly larger scale than in the NH group, and the HA children were judged to be significantly more hypernasal in comparison with NH children. CONCLUSIONS: Despite the fact that a substantial number of the CI and HA children demonstrate normal (nasal) resonance quality, this aspect of speech production is still at risk for hearing impaired children.

Is cochlear implantation an effective treatment for Menière's disease?

OBJECTIVES: describe the results of a retrospective study of cochlear implantation (CI) in seven subjects with Menière's disease. METHODOLOGY: The subjects received either the Nucleus CI24RE(CA)/CI512 or the Advanced Bionics HiRes90K CI systems which use the ACE, MP3000, or HiRes S Fidelity 120 coding strategies. The audiometric measures included monosyllabic word lists (NVA) in quiet at 65 dB SPL and sentences in noise (LIST) at +10 dB SNR. The quality of life after implantation was assessed by means of the Nijmegen Cochlear Implant Questionnaire (NCIQ). RESULTS: After CI, the hearing of all subjects improved significantly (p < 0.001) as did their speech recognition (p = 0.018). Speech recognition in noise showed a mean improvement of 47%. The results were less clear for the treatment of vertigo associated with Menière's, as some patients continued to have vestibular attacks after implantation. On the NCIQ, subjects reported a mean quality of life after CI of 48.3%. CONCLUSIONS: This study clearly demonstrates that cochlear implantation is an adequate treatment of speech perception for subjects with Menière's disease who go on to develop bilateral severe to profound sensorineural hearing loss.

New reference values must be established for the Alberta Infant Motor Scales for accurate identification of infants at risk for motor developmental delay in Flanders.

BACKGROUND: The Alberta Infant Motor Scales (AIMS) is a reliable and valid assessment tool to evaluate the motor performance from birth to independent walking. This study aimed to determine whether the Canadian reference values on the AIMS from 1990-1992 are still useful tor Flemish infants, assessed in 2007-2010. Additionally, the association between motor performance and sleep and play positioning will be determined. METHODS: A total of 270 Flemish infants between 0 and 18 months, recruited by formal day care services, were assessed with the AIMS by four trained physiotherapists. Information about sleep and play positioning was collected by mean of a questionnaire. RESULTS: Flemish infants perform significantly lower on the AIMS compared with the reference values (P < 0.001). Especially, infants from the age groups of 4, 5, 6, 7, 8, 9, 10, 11, 12, 13 and of 15 months showed significantly lower scores. From the information collected by parental questionnaires, the lower motor scores seem to be related to the sleep position, the amount of play time in prone, in supine and in a sitting device. Infants who are exposed often to frequently to prone while awake showed a significant higher motor performance than infants who are exposed less to prone (<6 m: P = 0.002; >6 m: P = 0.013). Infants who are placed often to frequently in a sitting device in the first 6 months of life (P = 0.010) and in supine after 6 months (P = 0.001) performed significantly lower than those who are placed less in it. CONCLUSION: Flemish infants recruited by formal day care services, show significantly lower motor scores than the Canadian norm population. New reference values should be established for the AIMS for accurate identification of infants at risk. Prevention of sudden infant death syndrome by promoting supine sleep position should go together with promotion of tummy time when awake and avoiding to spent too much time in sitting devices when awake.

A pilot cost-of-illness study on long-term complications/sequelae of AOM.

OBJECTIVES: Acute otitis media (AOM) commonly affects young children and occasionally results in serious complications/sequelae. This pilot cost-of-illness study aimed to assess the economic burden of long-term AOM complications/sequelae in Belgium, and to establish a thorough methodology for a larger study. METHODOLOGY: We retrospectively reviewed charts of patients aged 10-20 years with long-term complications/sequelae considered to be AOM-related, and > or = 8 years of follow-up. From a list of 215 eligible patients, we selected 25 patients representing each of seven categories of complications/sequelae. RESULTS: Included patients had a mean age of 12.9 years; nine had chronic suppurative otitis media with cholesteatoma; six sensorineural hearing loss; six chronic perforation of the tympanic membrane; and one each with conductive hearing loss, facial paralysis, neurological impairment after intracranial complications, and complications of surgery. During 8-15 years of follow-up, the most common complications were hearing loss, chronic otitis media (OM), and cholesteatoma. These generally occurred > 5 years after the first AOM event, although chronic OM occurred after a mean time of 3.3 years. Yearly public health care payer (PHCP) costs ranged from Euro 119 to Euro 7957 per patient, and were highest for patients with sensorineural hearing loss. Yearly costs to the patients ranged from Euro 7 to Euro 289 per patient, and were also highest for patients with sensorineural hearing loss. CONCLUSIONS: Although complications/sequelae of AOM are rare, they can result in substantial costs. The applied methodology should be feasible for a larger study, with some minor adjustments.

Cochlear implantation in posttraumatic bilateral temporal bone fracture.

Temporal bone (TB) fractures can cause loss of audiovestibular function. Four cases of profound hearing impairment following bilateral TB fracture are presented in this report. All patients received a cochlear implant. All 4 patients became regular users of their implants. None of the patients experienced facial nerve stimulation. Implant-aided audiometry demonstrated a hearing threshold of 28 dB HL. The performance in speech understanding was comparable to standard postlingual adult patients implanted. We believe that cochlear implantation in patients suffering from profound sensorineural hearing losses secondary to TB fractures can be an effective tool for rehabilitation.

Basic principles for paediatric care: what ENT professionals should know.

Children undergoing medical or surgical treatment for ENT disorders should receive care from doctors familiar with the specificities of paediatric ENT pathology working in dedicated clinics where there are facilities for the activities that children usually indulge in and accommodation for parents. Many aspects of care for children with ENT problems involve a multidisciplinary team consisting of ENT surgeons working alongside a range of medical and paramedical professionals and nurses specifically trained in childcare, as well as in ENT nursing. Within this multidisciplinary approach, we will discuss some important aspects of the psychosocial approach and nursing, anaesthesia and pharmacotherapy that should be considered in order to raise the safety and quality of patient care in paediatric otorhinolaryngology.

Test-retest reliability of the assessment of postural stability in typically developing children and in hearing impaired children.

The purpose of this study was to establish test-retest reliability of centre of pressure (COP) measurements obtained by an AccuGait portable forceplate (ACG), mean COG sway velocity measured by a Basic Balance Master (BBM) and clinical balance tests in children with and without balance difficulties. 49 typically developing children and 23 hearing impaired children, with a higher risk for stability problems, between 6 and 12 years of age participated. Each child performed the modified Clinical Test of Sensory Interaction on Balance (mCTSIB), Unilateral Stance (US) and Tandem Stance on ACG, mCTSIB and US on BBM and clinical balance tests: one-leg standing, balance beam walking and one-leg hopping. All subjects completed 2 test sessions on 2 different days in the same week assessed by the same examiner. Among COP measurements obtained by the ACG, mean sway velocity was the most reliable parameter with all ICCs higher than 0.72. The standard deviation (SD) of sway velocity, sway area, SD of anterior-posterior and SD of medio-lateral COP data showed moderate to excellent reliability with ICCs between 0.55 and 0.96 but some caution must be taken into account in some conditions. BBM is less reliable but clinical balance tests are as reliable as ACG. Hearing impaired children exhibited better relative reliability (ICC) and comparable absolute reliability (SEM) for most balance parameters compared to typically developing children. Reliable information regarding postural stability of typically developing children and hearing impaired children may be obtained utilizing COP measurements generated by an AccuGait system and clinical balance tests.

A comparison of vowel productions in prelingually deaf children using cochlear implants, severe hearing-impaired children using conventional hearing aids and normal-hearing children.

OBJECTIVE: The purpose of this study was to compare vowel productions by deaf cochlear implant (CI) children, hearing-impaired hearing aid (HA) children and normal-hearing (NH) children. PATIENTS AND METHODS: 73 children [mean age: 9;14 years (years;months)] participated: 40 deaf CI children, 34 moderately to profoundly hearing-impaired HA children and 42 NH children. For the 3 corner vowels [a], [i] and [u], F(1), F(2) and the intrasubject SD were measured using the Praat software. Spectral separation between these vowel formants and vowel space were calculated. RESULTS: The significant effects in the CI group all pertain to a higher intrasubject variability in formant values, whereas the significant effects in the HA group all pertain to lower formant values. Both hearing-impaired subgroups showed a tendency toward greater intervowel distances and vowel space. CONCLUSION: Several subtle deviations in the vowel production of deaf CI children and hearing-impaired HA children could be established, using a well-defined acoustic analysis. CI children as well as HA children in this study tended to overarticulate, which hypothetically can be explained by a lack of auditory feedback and an attempt to compensate it by proprioceptive feedback during articulatory maneuvers.

Etiological diagnosis in the hearing impaired newborn: proposal of a flow chart.

OBJECTIVE: Most industrialized countries have introduced some form of universal newborn hearing screening program. Both identification and rehabilitation of hearing loss in newborns have evolved to an acceptable standard and the need for a standardized etiological protocol is emerging. METHODS: Extensive literature search to determine which investigations can help identifying the cause of congenital hearing loss and how to limit extensive testing in these children by taking into account the most prevalent causes. FINDINGS: A stepwise approach to detect the cause of hearing loss in children with congenital sensorineural hearing loss was developed. CONCLUSION: In general it is advised to first rule out Cx26/Cx30 and infectious causes (cytomegalovirus and, if indicated, toxoplasmosis and rubella), and to preserve more extensive investigations for those children in whom these causes do not explain the hearing loss.

The prevalence of middle ear pathogens in the outer ear canal and the nasopharyngeal cavity of healthy young adults.

Culturing middle ear fluid samples from children with chronic otitis media with effusion (OME) using standard techniques results in the isolation of bacterial species in approximately 30-50% of the cases. Haemophilus influenzae, Streptococcus pneumoniae and Moraxella catarrhalis, the classic middle ear pathogens of acute otitis media, are involved but, recently, several studies suggested Alloiococcus otitidis as an additional pathogen. In the present study, we used species-specific PCRs to establish the prevalence, in both the nasopharyngeal cavity and the outer ear, of H. influenzae, M. catarrhalis, S. pneumoniae and A. otitidis. The study group consisted of 70 healthy volunteers (aged 19-22 years). The results indicate a high prevalence (>80%) of A. otitidis in the outer ear in contrast to its absence in the nasopharynx. H. influenzae was found in both the outer ear and the nasopharynx (6% and 14%, respectively), whereas S. pneumoniae and M. catarrhalis were found only in the nasopharynx (9% and 34%, respectively).A. otitidis, described as a fastidious organism, were able to be cultured using an optimized culture protocol, with prolonged incubation, which allowed the isolation of A. otitidis in five of the nine PCR-positive samples out of the total of ten samples tested. Given the absence of the outer ear inhabitant A. otitidis from the nasopharynx, its role in the aetiology of OME remains ambiguous because middle ear infecting organisms are considered to invade the middle ear from the nasopharynx through the Eustachian tube.

Otitis media microbes: culture, PCR, and confocal laser scanning microscopy.

OBJECTIVES: To assess the presence of middle ear pathogens in nasopharynx (NP), middle ear fluid (MEF), and middle ear mucosal swabs (MES) of 14 patients undergoing middle ear surgery. METHODOLOGY: Bacteria were assessed by culture and species specific PCR. Biofilm was investigated by confocal laser scanning microscopy (CLSM) of middle ear biopsies (MEBs). RESULTS: Bacteria were absent in CLSM of MEBs in three of the four closed and healthy middle ears. Bacteria occurred in the ear with a foreign body (middle ear prosthesis), which showed localized living and dead bacteria, indicating biofilm. Bacterial growth was present in ten patient ears, but biofilm occurred in only one patient. CLSM indicated biofilm in the middle ear of two patients for whom PCR detected Haemophilus influenzae in the MEF. The three classical pathogens could frequently be found in the nasopharynx, by culture and PCR, but not from the middle ear. Alloiococcus otitidis was detected in the MEF of all five patients with open inflamed ears, though virtually absent from the nasopharynx. Pseudomonas aeruginosa was present in seven. It was the only pathogen found on several occasions in all three locations in one patient. CONCLUSIONS: This study confirms the association of H. influenzae with middle ear biofilm, and indicates a potential role of P. aeruginosa in middle ear inflammation and biofilm formation. Biofilm does not seem to cause inflammation. It is unclear whether the predominance of A. otitidis in chronically inflamed open middle ears indicates a pathogenic or contaminant role for this organism.

Cochlear implantation in 3 adults with auditory neuropathy/auditory dys-synchrony.

We describe 3 adult patients with auditory neuropathy/auditory dys-synchrony (AN/AD) who underwent cochlear implantation. All patients had absent or poorly formed auditory brainstem responses (ABRs) in combination with preserved otoacoustic emissions (OAEs). They exhibited various aetiologies and a large variation in clinical features known to be consistent with AN/AD. Cochlear implantation was successful in 2 out of 3 cases. We conclude that AN/AD implantee candidates should be counselled with care.

Evaluation of the rotatory vestibular test: exploration of stimulus parameters.

OBJECTIVES: The aim of this study was to construct a rotational test protocol after exploring the stimulus parameters of the rotatory vestibular test. METHODOLOGY: Twenty-four normal subjects were submitted to three different rotational paradigms: the sinusoidal harmonic acceleration test (SHAT), the pseudorandom rotation test (PRRT), and the velocity step test (VST). We investigated the influence of frequency and velocity on gain, phase and asymmetry values for the SHAT and the PRRT. In the case of the VST, we examined the influence of velocity and deceleration on gain, slow component velocity at deceleration, time constant, nystagmus preponderance, and time constant asymmetry. RESULTS: Frequency affected the SHAT response parameters, with significant phase differences between the frequencies 0.01, 0.02, 0.05, 0.1, and 0.2 Hz, while velocity, if kept below 150 degrees/s, had no influence on the results. In the case of the VST, responses were influenced by stimulus velocity and not by stimulus deceleration, with significantly higher gain values for the velocities 50 and 250 degrees/s. CONCLUSIONS: A velocity of 50 degrees/s tested at the frequencies 0.01, 0.02, 0.05, 0.1, and 0.2 Hz was suggested for the SHAT and PRRT protocol, whereas a velocity of 100 degrees/s with a deceleration of 200 degrees/s2 was preferred for the VST. The relevance of this rotational protocol has yet to be established from patient data.