Screening for delayed-onset hearing loss in preschool children who previously passed the newborn hearing screening

Objective

The goal of this study was to investigate the prevalence of delayed-onset hearing loss in preschool children who previously passed newborn hearing screening in Shanghai, China.

Methods

Between October 2009 and September 2010, 21,427 preschool children were enrolled from five areas of Shanghai, who had passed newborn hearing screening. Children were screened for delayed-onset hearing loss with pediatric audiometers. Children with positive results in initial and re-screening tests were assessed audiologically and for risk indicators according to the Year 2007 Statement of the American Joint Committee on Infant Hearing (JCIH).

Results

During the study period, 445 children (2.08%) were referred for audiologic assessment; 16 (0.75/1000, 95% CI 0.38-1.12) had permanent delayed-onset hearing loss. Of these, five (0.23/1000) had bilateral moderate hearing loss; seven (0.33/1000) had mild bilateral hearing loss; and four (0.19/1000) had unilateral moderate or mild hearing loss. JCIH risk indicators were established for six children (6/16, 37.5%), including parental concern regarding speech and language developmental delay, neonatal intensive care unit with assisted ventilation or hyperbilirubinemia, recurrent otitis media with effusion, craniofacial malformation, and family history (n = 1 for each). The remaining ten (62.5%) had no related risk factors.

Conclusions

A significant proportion of preschool children have undiagnosed delayed-onset hearing loss. Hearing screening in preschool is recommended for an early detection.     

The audiological characteristics of a hereditary Y-linked hearing loss in a Chinese ethnic Tujia pedigree

Objective

To investigate audiometric characteristics of hearing loss in a large Chinese ethnic Tujia family and determine its hereditary type.

Methods

Total 76 live individuals were investigated in the notable 84 members of this family. The detailed audiometric evaluations were undertaken for the proband and his 47 family members. The degrees of sensorineural hearing impairment were defined as an air/bone gap <15 dB hearing loss averaged over 0.5, 1 and 2 kHz. The severity of hearing loss was established based on the hearing ability of the better ear, averaged over 0.5, 1, 2 and 4 kHz, and classified into four categories: mild, moderate, severe and profound.

Results

Nineteen patrilineal relatives of the 76 live members had hearing impairment. The age of onset ranged from 7 to 21 years old with the average of 13.2 years. The audiometric defect was described by auditory curves of a high frequency in 47% of the patients. Affected members in this family demonstrated a non-syndromic, late onset, bilateral, symmetrical, postlingual and sensorineural hearing loss.

Conclusions

The audiometric configuration in males of the pedigree is consistent with the hereditary Y-linked hearing loss. Thus we speculate that a putative gene on the Y chromosome could contribute to the cause of the disease.     

Prognostic indicators for sensorineural hearing loss in temporal bone histiocytosis

Objective

Our review aims: (1) to highlight the suspicion of sensorineural hearing loss (SNHL) in temporal bone Langerhans’ cell histiocytosis (LCH); (2) to assess the evolution of SNHL in this pathology; (3) to identify radiologic findings of the otic capsule invasion by LCH; (4) to determine prognostic factors for SNHL in temporal bone LCH.

Methods

We performed a literature review through MEDLINE for SNHL in temporal bone LCH related articles that were published between 1954 and 2008. We identified 12 related studies of which 18 patients were noted. We also added our case to this series. The information from the reports was analyzed to characterize the clinical and demographic data and to focus on the sensorineural hearing aspect of the disease and on the damage of the semicircular canals (SCC) and the cochlea.

Results

Ten percent of patients with temporal bone LCH presented SNHL. The mean age of patients is 3.5 years among children and 35.5 years among adults. Male to female ratio is 1:1.14. There were 13 unilateral cases, 6 bilateral cases and 1 case of multisystemic histiocytosis. Cochlea and SSCs were involved in 4 and 20 temporal bones, respectively. The lateral SCC is the most frequently eroded canal. In 23 ears hearing level was reported: 15 ears had a SNHL and the remaining was a mixed or a conductive hearing loss type. 10 ears suffered from a profound hearing loss and none of them improved after treatment regardless otic capsule affected structures. In all cases of normal hearing, moderate hearing loss and severe hearing loss before treatment cochlea have not been affected. However a single or two semicircular canals invasions have been noted. Where it is reported (15 out of 25 temporal bone) there was a radiological healing of the otic capsule lesion after treatment. Remineralization occurs 6 months after treatment.

Conclusion

In LCH, hearing loss level before treatment can be considered as a prognostic indicator for hearing in response to treatment. Even though bone mineralization is essential for a hearing improvement, radiological healing is not a prognostic factor for better hearing level after treatment.     

Eustachian tube function in patients with Meniere's disease

Objective

The aim of the present study was to clarify the relationship between Eustachian tube function and inner ear function, especially with respect to the hearing ability of patients with Meniere's disease.

Methods

Patients with Meniere's disease underwent nystagmic examinations and audiometric measurements, including hearing tests, tympanometry, and Eustachian tube function tests (sonotubometry). We compared the audiometric examination results of normal subjects to those of patients with Meniere's disease.

Results

Twenty-five percent of patients with Meniere's disease exhibited Eustachian tube dysfunction, but 92% displayed normal tympanometry findings. Their sonotubometry durations and amplitudes were not significantly different from those of normal subjects. However, the patients’ hearing level was significantly correlated to sonotubometry duration and amplitude. Our patients were classified according to the four stages of Meniere's disease: stage 1 (n = 9); stage 2 (n = 5); stage 3 (n = 8); and stage 4 (n = 2). The incidence of Eustachian tube dysfunction in these four groups of patients were 0% (0/9); 40% (2/5); 38% (3/8); and 50% (1/2), respectively.

Conclusion

Our study provides evidence demonstrating that treatment of Eustachian tube dysfunction may be useful in preventing the hearing of Meniere's patients from becoming worse.     

Audiological chronological findings in children with congenital anomalies of the central nervous system

Objective

To determine the frequency of hearing impairment in children with congenital anomalies of the central nervous system (CNS) by using detailed audiological evaluation methods.

Methods

The patients were 78 children with congenital anomalies of the CNS with a mean age of 29.5 months. They had been observed for a mean period of 38.5 months. Hearing levels were evaluated behavioral observation audiometry (BOA), visual reinforcement audiometry (VRA) and distortion product otoacoustic emissions (DPOAEs) were performed. Auditory brainstem responses (ABRs) and computed tomography (CT) scans of the temporal bone were performed in the cases in which the minimum response levels (MRLs) were above 30 dBHL. All cases were assessed in terms of developmental age.

Results

A total of 14.1% (11/78) of the children with congenital anomalies of the CNS were initially diagnosed with bilateral sensorineural hearing loss (SNHL). However, the hearing levels of nine of them improved by the time of the last diagnosis. Therefore, the patients with bilateral SNHL were only 2.6% (2/78) of the total patients with congenital anomalies of the CNS at last diagnoses. As shown by our results, many children with bilateral SNHL at initial diagnosis showed improved ABR thresholds and behavioral hearing thresholds with age. In this series, the use of hearing aids was arranged for six patients. However, four patients stopped using hearing aids when their hearing threshold levels improved. In two cases, there were no changes in hearing levels and the children continued using hearing aids.

Conclusion

Our results suggest that hearing level recovery can occur in some children with CNS anomalies. Confirmation of hearing loss in children with congenital anomalies of the CNS takes a long time. There are improvements in hearing loss during the observation period. Therefore periodic assessment of hearing is important.     

Implementation of neonatal screening for hearing impairment: Influence on pediatric otitis media surgery in The Netherlands

Objective

Screening for hearing impairment in the Netherlands took place at the age of 9 months for many years but was recently moved to the neonatal period. Since otitis media has its highest prevalence at the age of 9 months, it might be that screening for hearing impairment at this age is linked to treatment of otitis media. We were interested in what the impact would be on the number of children treated with ventilation tubes or adenoidectomy when they were no longer screened at the age of 9 months.

Methods

National birth rates, data regarding screening for hearing impairment at the age of 9 months and at the neonatal period, as well as data regarding adenoidectomy and tube placement were used to study treatment of otitis media in children aged 0-2 years in the Netherlands.

Results

The percentage of children treated with ventilation tubes after implementation of neonatal screening for hearing impairment did not decline (OR 1.198-1.112), and more children were treated at a younger age. The number of children treated with adenoidectomy did however decline (OR 0.724-0.890).

Conclusion

There seems to be an association between the implementation of neonatal screening for hearing impairment and the treatment of otitis media. During the whole studied period there was a slight but statistical significant increase in the odds for tube placement in all children aged 0-23 months. After implementation of the neonatal screen there was a distinct increase in the number of children aged 6-11 months treated with tubes. In the same period a statistical significant decline in the odds of undergoing adenoidectomy was observed.     

Non-organic hearing loss in childhood

Objective

The present study aimed to investigate the etiology, symptoms, diagnosis and prognosis of pediatric patients with non-organic hearing loss (NOHL), and to heighten awareness of this disorder among physicians.

Methods

Between January 2000 and July 2009, we retrospectively reviewed the medical records of 47 pediatric patients (aged 6-18 years of age) diagnosed with NOHL. The diagnosis was made when there were audiometric discrepancies between the subjective and objective hearing thresholds of the patient in the absence of any organic disease.

Results

Eighteen patients presented with unilateral hearing loss, and 29 showed bilateral hearing loss. Five patients received steroid treatment before the correct diagnosis was made, and six had secretory otitis media and underwent a tympanostomy tube placement.

Conclusion

If physicians are unaware of the possibility of NOHL; they may misdiagnose children with idiopathic sudden sensorineural hearing loss and administer high-dose steroid treatments or exploratory tympanotomies. Otoacoustic emissions are abolished when NOHL patients have secretory otitis media. In these cases, after tympanostomy tube placement, they should undergo objective electrophysiologic examinations to reevaluate NOHL.     

Appropriate hearing screening in the pediatric patient with head trauma

Purpose

To develop an algorithm for the appropriate audiologic screening of in children with head trauma.

Methods

Participants were the first consecutive 50 children admitted to a children's hospital trauma service after October 1, 2005, whose injuries resulted in a Glasgow Coma Scale (GCS) score ≤13 and/or loss of consciousness (LOC) but no history of hearing loss. Screening tympanometry, otoacoustic emissions testing, and/or routine audiometric evaluation were performed as soon as possible after admission. Age, GCS score, Pediatric Trauma Score, Injury Severity Score, presence of head and neck soft tissue injury, temporal bone fracture, skull fracture not involving the temporal bone, midface/mandible fractures, intracranial abnormality on computed tomography, and cervical fracture were analyzed as risk factors for hearing loss.

Results

Seventeen (34%) of the 50 children had abnormal hearing test results. Fischer's exact test showed abnormal test results were most strongly related to temporal bone fracture (p = 0.0041), non-temporal bone skull fracture (p = 0.0211) and younger age (p = 0.0638).

Conclusions

Any child with head trauma and clinical or radiologic evidence of temporal bone fracture should have early hearing evaluation. Using the proposed algorithm to test children with head trauma and GCS ≤13 and/or LOC and age <3 years or any type of skull fracture may help identify children with hearing loss in a more cost effective manner.     

Sensorineural hearing loss in pediatric celiac patients

Objective

Celiac disease (CD) is an immune-mediated chronic inflammatory gluten-dependent intestinal disease affecting 0.5-1% of the general population worldwide. CD is underdiagnosed even with sophisticated health care; approximately 10% of people affected by CD are now diagnosed. The recognition of the atypical extra-intestinal manifestations, including neurological disorders increased the diagnosis of CD. At present, no data are available on the presence of sensorineural hearing loss in pediatric CD patients. The aim of this study was to determine the incidence and severity of sensorineural hearing loss (SNHL) in different frequencies in pediatric CD patients.

Methods

A sample of 32 biopsies and serologically proven newly diagnosed pediatric CD patients (CD group) (64 ears) and 32 sex and age-matched healthy subjects (64 ears) as control group (C group) were included in this study. Anthropometric measurements, physical examinations including ear nose and throat and pure-tone audiometry at frequencies 250-8000 Hz were performed in all subjects in both groups. Slight/mild SNHL was defined as a loss of detection of sound within the 16-40 dB range. The mean age of patient and control group was 11.9 and 11.3, respectively (p > 0.05).

Results

In CD group, sensorineural hearing loss was found in 13 (40.6%) patients (group A) as it was bilateral in six and unilateral in seven patients. In control group (group C), slight/mild SNHL was found in one (3.1%) subject. The frequency of hearing loss was significantly higher in CD group than in group C (p < 0.001).

Conclusion

The present study showed a higher prevalence of sensory neural hearing loss in pediatric celiac patients than in healthy controls, suggesting an association between CD and SNHL. The findings of this study suggest that hearing impairment should be searched in newly diagnosed pediatric CD patients. Further longitudinal investigations on a larger sample size will be necessary to confirm the present data and to search the immunological processes which could be the basis of the association between CD and SNHL.     

Hearing assessment in high-risk congenital diaphragmatic hernia survivors

Objective

To report results of audiometric evaluations in high-risk congenital diaphragmatic hernia survivors and their exposure to audiological risk factors (mechanical ventilation, high frequency oscillation, aminoglycoside therapy and neuromuscular blocking agents).

Design

All newborns with high-risk congenital diaphragmatic hernia born between January 2003 and June 2009 were treated consecutively at the Neonatal Intensive Care Unit, Pediatric Hospital, University of Padova. Thirty-two survived and 26 of them underwent formal audiological evaluation (tonal and speech audiometry, otoacoustic emission, and immitance measurements) and follow up.

Results

Twenty-one children had normal hearing; 4 had conductive hearing loss, which was successfully treated; and 1 had severe sensorineural hearing loss and suffers from Turner syndrome.

Conclusions

Our series revealed a lower prevalence of sensorineural hearing loss in high-risk congenital diaphragmatic hernia survivors than in other studies, suggesting that the association between hearing loss and congenital diaphragmatic hernia has yet to be accurately defined and fully elucidated.     

Auditory function in women with autoimmune inner ear diseases and their offspring

Objective

The precise cause of congenital sensorineural hearing loss (CSNHL) is unclear in many cases. In a previous study we found that offspring from guinea pigs with autoimmune sensorineural hearing loss (ASNHL) exhibited signs of SNHL. Here we studied women with autoimmune inner ear diseases (AIED) and their offspring. Our aim was to determine if autoimmune damage may be one of the causes of CSNHL.

Methods

Thirty-eight pregnant women with AIED were recruited. Thirty-three had ASNHL; one with autoimmune delayed endolymphatic hydrops (ADEH) and four with autoimmune Meniere's disease (AIMD). The following were assessed in all women: audiogram, auditory brain stem response (ABR), otoacoustic emission (OAE), vestibular function test and presence of inner ear antigens. The following were assessed in offspring from these women: OAE, ABR and presence of inner ear antigens.

Results

Five of the 38 children born to women with AIED had SNHL (an incidence much higher than normal). OAEs were not inducible in these children shortly after birth or within 46-100 days after birth. Abnormal ABR findings were apparent in these five children and inner ear antigens were detected in three of the five children (the mother's of these children were also positive for inner ear antigens).

Conclusions

These preliminary findings suggest that the prevalence of congenital ASNHL may be increased in offspring born to women with AIED.     

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 role of dyslipidemia in sensorineural hearing loss in children

Objective

The relationship between dyslipidemia and hearing is controversial; especially in children, the available evidences are scarce. This study is designed to determine whether dyslipidemia is associated with sensorineural hearing loss in a group of 5-18-year-old children and adolescents.

Methods

Through an analytic cross-sectional study, records of all 5-18-year-old children who attended the pediatric endocrinology clinic of Loghman Hospital in Tehran, Iran, between April 2007 and April 2009 were reviewed. Records with a lipid profile were determined. Lipid profiles were repeated for eligible cases; they were enrolled if the results were the same as before (normal or dyslipidemic). Hearing loss, speech discrimination scores and lipid profiles were analyzed.

Results

When controlled for age and sex, no association between dyslipidemia and sensorineural hearing loss were found. There was also no statistically significant relationship between dyslipidemia and sensorineural hearing loss in different age groups.

Conclusions

Dyslipidemia seems to have no association with sensorineural hearing loss in 5-18 years old children according to this study.     

Place of birth and characteristics of infants with congenital and early-onset hearing loss in a developing country

Objective

To determine the characteristics of infants with sensorineural hearing loss (SNHL) and the relationship with place of birth.

Methods

Subjects were drawn from hospital-based and community-based universal infant hearing screening programs concurrently conducted from May 2005 to April 2008 in Lagos, Nigeria. Maternal and infant characteristics of children born in hospitals and detected with SNHL were compared with those born outside hospitals. Each program consisted of a first-stage screening with transient-evoked otoacoustic emissions (TEOAE) followed by second-stage automated auditory brainstem response (AABR). Hearing status was confirmed by diagnostic auditory brainstem response, tympanometry and visual response audiometry.

Results

A total of 4718 infants were screened under the hospital-based program out of which 12 (0.3%) infants were confirmed with SNHL whereas 71 (1.0%) of the 7179 infants screened under the community-based program were confirmed with SNHL. Of all infants with SNHL 39 (47.0%) were born in hospitals suggesting that 27 (38.0%) of infants under the community-based program were born in hospitals. Prevalence of SNHL ranged from 4.0 per 1000 among infants born in government hospitals to 23 per 1000 among those born in family homes. Mothers of those born outside hospitals were significantly likely to belong to the Yoruba tribe (p < 0.001), use herbal medications in pregnancy (p < 0.001), deliver vaginally (p = 0.004) but without skilled attendants at delivery (p < 0.001). There were no significant differences among the infants themselves except that those born outside hospitals were significantly likely to be detected in the first 3 months of life compared to those born in hospitals (p < 0.001).

Conclusions

A significant proportion of infants with SNHL in many developing countries are likely to be born outside hospitals thus underscoring the need for community-oriented UNHS to facilitate early detection and intervention. Conventional risk factors for SNHL are unlikely to discriminate across places of birth. Pediatricians and otolaryngologists should consider a more active role in fostering community-oriented delivery of primary ear care services in this and similar settings in the developing world.     

Cochlear involvement in Familial Mediterranean Fever: a new feature of an old disease

Objectives

In this study we first aimed to assess the cochlear functions in children with Familial Mediterranean Fever. The second aim was to investigate the correlation between the hearing levels and some clinical features of Familial Mediterranean Fever including the duration of the disease, age at onset, genetic analysis and colchicine use.

Methods

Thirty-four children with Familial Mediterranean Fever and 27 age matched children were included in the study. Following otologic examination, all children underwent audiometric evaluation, including Pure Tone Average measurements and Distortion Product Otoaoustic Emission testing. Audiological results of the two groups were compared and correlation between the audiologic status and clinical parameters of the disease like the duration of disease, age at onset, mutations and colchicine treatment were studied.

Results

Pure tone audiometry hearing levels were within normal levels in both groups. Hearing thresholds of Familial Mediterranean Fever patients were found to be increased at frequencies 8000, 10,000, 12,500 and 16,000 (p < 0.05). In otoacoustic emission evaluation, distortion products and signal-noise ratio of FMF children were lower in the tested frequencies, from 1400 Hz to 4000 Hz (p < 0.05). Interaction of the disease duration and age of disease onset was found to predict hearing levels, distortion products and signal-noise ratios of children with Familial Mediterranean Fever (F value = 2.034; p = 0.033).

Conclusions

To our knowledge this is the first study demonstrating cochlear involvement in children with Familial Mediterranean Fever which showed increased hearing thresholds at higher frequencies in audiometry together with decreased distortion products and signal-noise ratios demonstrated by distortion product otoacoustic emission testing. Similar studies must be carried out on adult patients to see if a clinical hearing impairment develops. The possible mechanisms that cause cochlear involvement and the effect of colchicine treatment on cochlear functions must be enlightened.     

Prevalence and etiology of congenital or early acquired hearing impairment in Eastern Finland

Objective

The purpose of this study was to determine the prevalence and etiology of congenital or early acquired bilateral sensorineural hearing impairment (SNHI) in children born from 1988 to 2002 in the district of Kuopio University Hospital, Finland, and to compare the results with those from an earlier 14-year period in the same region and similar population.

Methods

The data were collected retrospectively from Hospital Records. The degree of hearing impairment was based on average air conduction threshold calculated over the frequencies 0.5, 1, 2 and 4 kHz in the better hearing ear. Hearing impairment was classified as mild (≥20-39 dB), moderate (40-69 dB), severe (70-95 dB) and profound (>95 dB).

Results

We identified 92 children with bilateral SNHI diagnosed before the age of 7 years. The overall prevalence and the prevalence for at least moderate SNHI was 2.1 per 1000 live births and 1.2 per 1000 live births, respectively. We found no differences in the prevalence of SNHI during both study periods. Etiology was genetic in 46%, acquired in 14% and unknown in 40%. Out of the genetic cases 74% were non-syndromic and 26% were syndromic. In comparison to the previous study there was a decline in the proportion of acquired SNHI and the proportion of genetic and unknown cause had increased. Six children in five families had homozygous 35delG mutation and six children in four families presented with a homozygous M34T mutation.

Conclusions

The prevalence of congenital or early acquired SNHI in the Kuopio University Hospital district area has not changed during a period of 29 years. Despite possibility to test the GBJ2 gene, the proportion of hearing impairment of unknown etiology remained high.     

Congenital incudostapedial anomalies in adult stapes surgery: a case-series review

Objectives

The management of developmental anomalies of the ossicular chain and otic capsule in stapes surgery tests the otologist's diagnostic skills and flexibility in technique and prosthesis choice. We review our experience in managing various incudostapedial developmental anomalies discovered during middle ear exploration for suspected otosclerosis in adult patients.

Design

A retrospective case-series review was used.

Setting

The study was set at a military tertiary referral center.

Patients and other Participants

A review of 185 stapes procedures yielded 8 cases of identified congenital anomalies of the stapes or incudostapedial complex including congenital absence of the oval window with malformed incus and stapes, columellar-type stapes with promontory fixation, stapes superstructure fixation to the fallopian canal, and malformed incus with stapes superstructure fixation to the promontory.

Interventions

The developmental anatomy and surgical technique in each case were reviewed. The surgical technique used in each case varied depending on the specific ossicular abnormality found and whether both the stapes and incus were affected.

Main outcome measures

Comparison of preoperative and postoperative audiometric testing results was performed.

Results

All 8 ears (7 patients) that underwent a stapes procedure for either an isolated congenital stapes anomaly or a combined incudostapedial anomaly had a favorable hearing outcome, with 7 ears improving to within 10-dB and 1 ear to within 13-dB air-bone gap on postoperative audiometry.

Conclusions

Despite unanticipated findings of congenital anomalies of the stapes and incus during middle ear exploration for conductive hearing loss, a flexible management approach can lead to successful hearing outcomes.     

An initial overestimation of sensorineural hearing loss in NICU infants after failure on neonatal hearing screening

Objective

Infants admitted to neonatal intensive care units have a higher incidence of significant congenital hearing loss. We classified audiologic diagnoses and follow-up in infants who had been admitted to our neonatal intensive care unit.

Methods

We included all infants admitted to the neonatal intensive care unit at Sophia Children's Hospital between 2004 and 2009 who had been referred for auditory brainstem response measurement after failing neonatal hearing screening with automated auditory brainstem response. We retrospectively analyzed the results of auditory brainstem response measurement.

Results

Between 2004 and 2009 3316 infants admitted to our neonatal intensive care unit had neonatal hearing screening. 103 infants failed neonatal hearing screening: 46 girls and 57 boys. After first auditory brainstem response measurement we found 18% had normal hearing or a minimal hearing loss. The remainder had a type of hearing loss, distributed as follows: 15% conductive, 32% symmetric sensorineural, 14% asymmetric sensorineural, and 21% absent auditory brainstem responses. Repeated auditory brainstem response measurement showed a shift in hearing outcome. The main difference was an improvement from symmetric sensorineural hearing loss to normal hearing. However, in a small percentage of children, the hearing deteriorated.

Conclusions

As many as 58% of infants in this high-risk population who failed the neonatal hearing screening were diagnosed with sensorineural hearing loss or absent auditory brainstem responses. An initial overestimation of sensorineural hearing loss of about 10% was seen at first auditory brainstem response measurement. This may be partially explained by a conductive component that has resolved. Finally, in a small percentage of children the hearing deteriorated.