Behavioral Audiometry Research Articles

A Low Cost Setup for Behavioral Audiometry in Rodents

In auditory animal research it is crucial to have precise information about basic hearing parameters of the animal subjects that are involved in the experiments. Such parameters may be physiological response characteristics of the auditory pathway, e.g. via brainstem audiometry (BERA). But these methods allow only indirect and uncertain extrapolations about the auditory percept that corresponds to these physiological parameters. To assess the perceptual level of hearing, behavioral methods have to be used. A potential problem with the use of behavioral methods for the description of perception in animal models is the fact that most of these methods involve some kind of learning paradigm before the subjects can be behaviorally tested, e.g. animals may have to learn to press a lever in response to a sound. As these learning paradigms change perception itself (1,2) they consequently will influence any result about perception obtained with these methods and therefore have to be interpreted with caution. Exceptions are paradigms that make use of reflex responses, because here no learning paradigms have to be carried out prior to perceptual testing. One such reflex response is the acoustic startle response (ASR) that can highly reproducibly be elicited with unexpected loud sounds in naïve animals. This ASR in turn can be influenced by preceding sounds depending on the perceptibility of this preceding stimulus: Sounds well above hearing threshold will completely inhibit the amplitude of the ASR; sounds close to threshold will only slightly inhibit the ASR. This phenomenon is called pre-pulse inhibition (PPI) (3,4), and the amount of PPI on the ASR gradually depends on the perceptibility of the pre-pulse. PPI of the ASR is therefore well suited to determine behavioral audiograms in naïve, non-trained animals, to determine hearing impairments or even to detect possible subjective tinnitus percepts in these animals. In this paper we demonstrate the use of this method in a rodent model (cf. also ref. (5)), the Mongolian gerbil (Meriones unguiculatus), which is a well know model species for startle response research within the normal human hearing range (e.g. (6)).

Zur Verlässlichkeit hirnstammaudiometrischer Hörschwellenbestimmungen

Brainstem electric response audiometries (BERA) are in clinical use for a number of years. The aim of our study was to evaluate data regarding the long-term reliability of BERA-determined frequency specific thresholds in hearing disabled children. In a group of 97 hearing disabled children we sought to compare Notched-Noise- (NN) BERA threshold as well as Click-BERA thresholds taken shortly after birth with behavioral audiometry thresholds determined after 3.2 years (mean). We found a significant correlation between both BERA methods and the behavioral tests. However, the correlation coefficients for NN-BERA were higher than for Click-BERA thresholds. Our results provide evidence for a high reliability of the NN-BERA for characterization of early onset hearing disabilities in children. Our data suggest that pathologic findings in the Click-BERA should always be followed by a frequency specific analysis with NN-BERA.

Cochlear implantation in children with cochlear aplasia

Conclusion: Children with cochlear aplasia can achieve favorable speech perception abilities from cochlear implantation (CI) by inserting an electrode array into the vestibule if an audiological response is reliably demonstrated before surgery by behavioral audiometry and objective measures. Objectives: The purpose of this study was to evaluate the surgical outcome of CI in children with cochlear aplasia. Methods: The medical records of 454 children who underwent CI at the Dong-A University Hospital between January 1994 and September 2011 were reviewed. The prevalence of cochlear aplasia and the preoperative evaluations, operative findings, and postoperative speech perception abilities of implanted children with cochlear aplasia were evaluated. Results: The implanted children with cochlear aplasia accounted for 2% of those with inner ear malformations and 0.4% of all pediatric cochlear implantees. They showed audiological responses in preoperative electrophysiologic tests, and the eighth nerve was present in magnetic resonance imaging. The electrode array was inserted into the vestibule through an oval window after removing the stapes or through a transmastoid labyrinthotomy site. Electrical compound action potential and electrical auditory brainstem response tests showed clear neural responses after implantation. The hearing thresholds of the implanted ear improved to 25 dB HL and the children achieved significant open set speech perception abilities.

Hearing loss in skeletal dysplasia patients

A hearing screening program was performed to determine the prevalence of hearing loss and abnormal tympanometry in individuals with short-stature skeletal dysplasias attending a national meeting. Behavioral audiometry, otoacoustic emission testing, and tympanometry were used to assess hearing. Failed hearing screen was defined as hearing ≥ 35 dB at one or more frequencies or by "fail" on otoacoustic emissions. One hundred ten of 112 subjects completed the screening. 58 (51.8%) were children. Seventy-three (65.2%) had achondroplasia, 34 (30.4%) had one of 11 other diagnoses, and 5(4.4%) were undiagnosed. 25.8% of children failed hearing screening in one or both ears, while 46.3% of adults failed in one or both ears. 55.1% of adults and 25.0% of children with achondroplasia failed screening. Abnormal hearing was also found in the some patients with spondyloepiphyseal dysplasia congenital (SEDC; 75%), diastrophic dysplasia (66%), and Morquio (66%). Hearing was normal in those with hypochondroplasia, pseudoachondroplasia, and microcephalic osteodysplastic primordial dwarfism. Tympanometry was abnormal in at least one ear in 53.3% of children and 38.5% of adults. Abnormal tympanometry in the absence of functioning tympanostomy tubes was associated with 9.5 greater odds of hearing loss in children and 2.8 greater odds of hearing loss in the total cohort. Only 3 (2.7%) respondents reported the use of hearing aids. Hearing loss and middle ear disease are common in both children and adults with skeletal dysplasia. Adults were more likely to fail hearing screening than children. Abnormal tympanometry is associated with hearing loss. Hearing screening with appropriate intervention is recommended for these patients.

Audiological findings in Williams syndrome: A study of 69 patients

The aim of this study was to investigate, in a clinical setting, the auditory function of a group of individuals affected by Williams syndrome (WS). Sixty-nine patients with WS, aged 2-30, underwent comprehensive audiological testing including air/bone conduction behavioral audiometry, speech audiometry, tympanometry and measurement of the acoustic reflex, transient evoked otoacoustic emissions and brainstem auditory evoked responses. Hearing loss, defined by a pure-tone average above 15 dB HL, affected 22.6% of the patients studied with traditional audiometry and was mostly slight in severity. Hearing loss was conductive in 9.4% of patients, mainly children with otitis media with effusion, and sensorineural in 13.2% of patients. However, 30% of the ears studied had a hearing impairment in the high frequency range (high-frequency pure-tone audiometry above 15 dB HL), higher in participants above 15 years (46.15%) than in the younger ones (23.45%). Contralateral stapedial reflexes were present in all patients with A-type tympanograms. Transient otoacoustic emissions were absent in 44% of the ears of patients with normal hearing. Brainstem auditory evoked responses fell within normal ranges thus confirming the absence of retrocochlear dysfunction. Although hearing loss does not seem to be frequent, a cochlear fragility, especially in the high frequency range, related to outer hair cells is characteristic of WS. Therefore we strongly recommend monitoring patients affected by WS using annual audiometric tests and performing otoacoustic emissions in order to identify a subclinical cochlear dysfunction which might benefit from an audiological follow up before the possible onset of hearing loss.

National deafness program and behavioral enforcement audiometry

National deafness program and behavioral enforcement audiometry

Testing Babies: You Can Do It! Behavioral Observation Audiometry (BOA)

Behavioral observation audiometry (BOA) is the only test protocol that provides a direct measure of hearing. This valuable technique can be used to monitor hearing and hearing aid benefit in infants who are not yet able to participate in visual reinforcement audiometry (VRA). In this article, I present a carefully developed protocol that uses changes in sucking, resulting in a reliable threshold measures. Key points include Auditory brainstem response (ABR) testing, auditory steady state response (ASSR) testing, and otoacoustic emissions (OAEs) testing provide critical information about the status of the auditory pathways but are not direct measures of hearing. Only behavioral testing provides a direct measure of hearing. Behavioral testing can be used to monitor hearing and performance with hearing technology. When carefully performed, using appropriate criteria including using changes in sucking as an indication of a response, behavioral observation audiometry can be used by clinicians to accurately measure thresholds in infants cognitively less than 6 months of age.

Cochlear implantation in a child with auditory neuropathy spectrum disorder

ObjectiveTo report outcomes of cochlear implantation (CI) in a child with auditory neuropathy spectrum disorder (ANSD) and to provide preliminary clinical evidence of the efficacy of CI in ANSD patients. MethodsA 4–year–old boy with diagnosed auditory neuropathy spectrum disorder (ANSD) received implantation of a Nucleus CI24R after an unsatisfactory trial of amplification. Post–implantation performance in both hearing sensitivity and speech recognition was assessed in different sessions. Aided hearing thresholds were tested by behavioral audiometry. Mandarin Early Speech Perception Test (MESP), Meaningful Auditory Integration Scale (MAIS), category of auditory performance (CAP) and Speech Intelligibility Rating (SIR) were used to assess the benefits in auditory skills or speech recognition the boy obtained from CI. The tests were administered before surgery and at 3 months and 7 months after opening. ResultsThe boy demonstrated improved auditory sensitivity by using CI. Concerning speech recognition and communication, both speech audiometry and questionnaires showed an obvious benefit from CI. ConclusionsCI has worked efficiently in this ANSD boy. But because of limited understanding of ANSD and rehabilitation effect by cochlear implantation in this condition, the clinical decision to implant should be cautious and only after a thorough evaluation. Meanwhile, well controlled and long term studies are needed to confirm the efficacy of cochlear implantation in patients with ANSD.

Objective Assessment of Hearing in Children: Update on Procedures and Protocols

Early identification and diagnosis of hearing loss in infants and young children is the first step toward appropriate and effective intervention and is critical for optimal communicative and psychosocial development. Limitations of behavioral assessment techniques in pediatric populations necessitate the use of an objective test battery to enable complete and accurate assessment of auditory function. Since the introduction of the cross-check principle 35 years ago, the pediatric diagnostic test battery has expanded to include, in addition to behavioral audiometry, acoustic immittance measures, otoacoustic emissions, and multiple auditory evoked responses (auditory brainstem response, auditory steady state response, and electrocochleography). We offer a concise description of a modern evidence-based audiological test battery that permits early and accurate diagnosis of auditory dysfunction.

Is behavioral audiometry achievable in infants younger than 6 months of age?

Background and goal When carried out in addition to objective tests, behavioral audiometry performed in children with the so-called “Delaroche protocol” [IJORL 68 (2004) 1233-1243] enables to determine hearing thresholds by air and bone conduction over the whole auditory frequency range. In the present report, seventy-three hearing-impaired infants with different levels of motor and cognitive development were tested behaviorally before 6 months of age. Reliability of these early determined behavioral thresholds was then after analyzed using: (a) cross-sectional study, and (b) longitudinal study. Methods Cross-sectional study compared click-evoked ABR thresholds in the better ear with binaural high-frequency hearing thresholds. In longitudinal study, early measured binaural hearing thresholds from 500 through 4000 Hz were reassessed at 18 months. Results In 13% of babies behavioral testing was not fully completed by 6 months of age. Nevertheless, both cross-sectional and longitudinal studies yielded intraclass correlation coefficients above 0.80, suggesting that behavioral testing is applicable to this very young population. Conclusions Assessment of hearing after newborn screening should not be restricted to objective tests before 5½ months. It should also include bone- and air-conduction behavioral tests adjusted to developmental stage and performed in presence of parents.

Re-evaluation of CO 2 laser myringotomy for managing children with persistent acute otitis media

Background This study aimed to re-evaluate the utility of CO 2 laser myringotomy in children with persistent acute otitis media (PAOM). Methods From September 2002 to April 2008, 40 children with PAOM received CO 2 laser myringotomy. PAOM was defined as continuing symptoms and signs after systemic antibiotic treatment. Before laser myringotomy, the eardrums were checked under videotelescopy, pure tone audiometry or behavior audiometry and tympanometry. Middle ear effusions were collected using our own designed bottle culture device after laser myringotomy. The patients were followed up at outpatient clinics for 3 months. Cultured middle ear pathogens, healing time of the eardrums, hospital course (for admitted patients), and the development of middle ear effusions were recorded. Results Forty children with 53 ears with PAOM were enrolled. Eight patients (20%) had bilateral disease and underwent bilateral laser myringotomy. The overall culture rate was 28.3%, and Streptococcus pneumoniae was the most common pathogen. Eight patients (20%) were admitted for intravenous antibiotics. The average eardrum healing time was 22 days in those with positive cultures, and 16.4 days in those with negative cultures ( p = 0.125). All eardrums healed in 1 month. The resolution rate was 62.5% at 1 month. Patients with bilateral PAOM were prone to have positive middle ear culture (5/8) compared with those with unilateral PAOM (8/32) ( p = 0.086). Conclusion CO 2 laser myringotomy is an applicable means of treating PAOM. S pneumoniae is the most common pathogen in PAOM. CO 2 laser myringotomy allows for the drainage and culture sampling of middle ear effusion, relieving the need of taking prolonged medication.

Audiologic-Acoustic Evaluation of Speech Perception in Children with Cochlear Implants

An early diagnosis of the congenital disorder of hearing creates new challenges for a multidisciplinary team: paedoaudiologists, ear nose throat specialists, and speech therapists. The cross modality matching method is based on the objective and subjective techniques in the evaluation of hearing thresholds in children. The electrical response audiometry provides information about the response of the brainstem to acoustic stimulation; the behavioural audiometry gives information about the perception and central associative processes in the auditory pathway. The paediatric fitting procedure relies on solid foundations of behavioural measurement to ensure the validity of hearing aid and cochlear implant fitting. This study assessed perception of phonemes in children with the cochlear implants and possibilities of applying acoustic solutions to the audiologic evaluation. The authors have also examined the possibilities of applying digital audio processing algorithm in clinical practice. Self-developed computer controlled diagnostic stations were used and tested. Speech perception was assessed on the basis of Erber’s categories. Detection, discrimination and identification tests of 5 Ling phoneme were used. The sample comprised 23 implanted children, aged 3–6 years, who received a cochlear implant when they were 18 to 30 months old. The detection thresholds, discrimination and identification scores were assessed. Tests based on phonemes aa, uu, ii, ss, and sh (5 sounds of Ling) were used. The results indicated significant correlations between pure tone audiometry results and the thresholds of phoneme detection [dB SPL]. The identification score in this group was 95–100%.

F001 Temporary sensory hearing deficits after ear surgery – a retrospective analysis

To evaluate the auditory and speech abilities in children with auditory neuropathy spectrum disorder (ANSD) after cochlear implantation (CI) and determine the role of age at implantation.Ten children participated in this retrospective case series study. All children had evidence of ANSD. All subjects had no cochlear nerve deficiency on magnetic resonance imaging and had used the cochlear implants for a period of 12–84 months. We divided our children into two groups: children who underwent implantation before 24 months of age and children who underwent implantation after 24 months of age. Their auditory and speech abilities were evaluated using the following: behavioral audiometry, the Categories of Auditory Performance (CAP), the Meaningful Auditory Integration Scale (MAIS), the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS), the Standard-Chinese version of the Monosyllabic Lexical Neighborhood Test (LNT), the Multisyllabic Lexical Neighborhood Test (MLNT), the Speech Intelligibility Rating (SIR) and the Meaningful Use of Speech Scale (MUSS).All children showed progress in their auditory and language abilities. The 4-frequency average hearing level (HL) (500 Hz, 1000 Hz, 2000 Hz and 4000 Hz) of aided hearing thresholds ranged from 17.5 to 57.5 dB HL. All children developed time-related auditory perception and speech skills. Scores of children with ANSD who received cochlear implants before 24 months tended to be better than those of children who received cochlear implants after 24 months. Seven children completed the Mandarin Lexical Neighborhood Test. Approximately half of the children showed improved open-set speech recognition.Cochlear implantation is helpful for children with ANSD and may be a good optional treatment for many ANSD children. In addition, children with ANSD fitted with cochlear implants before 24 months tended to acquire auditory and speech skills better than children fitted with cochlear implants after 24 months.

Hearing Threshold Prediction Using Auditory Brainstem Response and Auditory Steady-State Response in Infants and Young Children

Received June 21, 2011 Accepted July 15, 2011 Address for correspondence Lee-Suk Kim, MD, PhD Department of OtolaryngologyHead and Neck Surgery, College of Medicine, Dong-A University, 1 Dongdaesin-dong 3-ga, Seo-gu, Busan 602-715, Korea Tel +82-51-240-5428 Fax +82-51-253-0712 E-mail klsolkor@chol.com Between one and three of every 1,000 neonates have sensorineural hearing loss (SNHL). It is of utmost importance to minimize the duration of auditory deprivation between the onset of bilateral deafness and intervention using hearing devices such as hearing aids and cochlear implants for achieving the best speech percpetion ability. To fit amplification accurately for children with SNHL, hearing thresholds for frequencies in the range of human communication should be evaluated. However, infants and young children are difficult to test using conventional behavioral tests, and hearing thresholds of them can be predicted using auditory brainstem response (ABR) and auditory steady-state response (ASSR). ABR is best evoked by applying a click stimulus, which allows an estimate over a broad range of high frequencies. ABR elicited by tone burst stimulus provides frequency-specific audiometric information. However, it can be difficult to record and observe at near-threshold levels, especially at lower frequencies. ABR thresholds for click and tone burst stimuli are highly correlated with behavioral thresholds, and often give an idea of the shape of an audiogram. ASSR is an auditory evoked potential, elicited with modulated tones. It provides frequency-specific hearing thresholds across the audiometric frequencies, which are well correlated with behavioral thresholds. However, the accuracy of threshold prediction decreases directly with the decrease of degree of hearing loss, and hearing thresholds cannot be predicted for auditory neuropathy. ASSR is most useful for estimating auditory thresholds for patients with no evidence of auditory neuropathy by the click ABR and OAEs, and who have an ABR only at high intensities or no ABR at a maximum stimulus level. Even if hearing thresholds are predicted through ABR and ASSR, behavioral testing including behavioral observation audiometry, visual reinforcement audiometry, or play audiometry should be employed repeatedly to verify the predicted thresholds, becasue the thresholds of ABR and ASSR are not true measure of hearing acuity but just responses generated at the brainstem. Korean J Otorhinolaryngol-Head Neck Surg 2011;54:592-602

わが国における乳幼児難聴のスクリーニングシステムとその課題

Childhood hearing impairment is a serious and relatively common condition. The earlier childhood hearing impairment is diagnosed, the less developmentally disadvantaged children become. Newborn hearing screening (NBS) programs have been implemented in Japan. NBS is important for identifying hearing loss at an early age and for adequate intervention at an early developmental stage. According to a survey questionnaire by the Japan Association of Obstetricians and Gynecologists, 62% of the newborn babies were audiologically and medically examined. The average age of examinees has become younger since the beginning of NBS. Here, we summarized the NBS programs in Japan including behavioral audiometry and examinations of auditory brainstem response, auditory-steady state response, and otoacoustic emissions. NBS can lead to advantages in terms of language developmental outcome for children with hearing impairment. However, there is no sufficient support system existing for children who are advised to undergo further auditory diagnostic tests after NBS. It is necessary for government agencies, medical and educational institutions to communicate together for clarifying their responsibilities in order to support the children with hearing impairment.

Treating options for deaf children

Background/Aim Although already established for metabolic diseases, universal screening programs for hearing have not been widely applied, despite the high incidence of profound congenital hearing loss. The present paper aims to review the current knowledge on the available treatment options for deaf infants. Data synthesis The acquisition of spoken language is a time-dependent process. For a child to become linguistically competent, some form of linguistic input should be present as early as possible in his/her life. Although objective audiological methods have certain weaknesses, their combination can give an accurate diagnosis in most of the cases. Later on, behavioural audiometry should confirm the diagnosis. Additional disabilities also need to be considered, although such assessments may be difficult in very young children. Congenital deafness should be managed by a multidisciplinary team (MDT). Affected infants should be bilaterally fitted with hearing aids, no later than three months after birth. They should be monitored and if they are not progressing linguistically, cochlear implantation (CI) should be considered after thorough preoperative assessment. Following CI, the vast majority of congenitally deaf children develop significant speech perception and production abilities over time. Age-at-intervention and oral communication, are the most important determinants of outcomes. Realistic parental expectations are also essential. The continuous support of a dedicated pediatric CI program, in collaboration with local professionals, and community members, are also necessary to achieve a successful outcome. Conclusion Congenitally deaf children should be detected early, and referred timely for the process of auditory rehabilitation to be initiated. Strong support by community members, and professional bodies, can maximize the future earnings of pediatric auditory rehabilitation with hearing aids and cochlear implants.

Delayed Recognition of Profound Hearing Loss in a 7-Year-Old Girl With a Neurological Condition

Kelly is a 7-year-old girl with a complex medical history including asthma, mild spastic diplegia, and seizure disorder that is controlled with carbamazepine. She had a significant receptive and expressive language impairment and milder delays in gross and fine motor skills. Kelly is currently repeating first grade in a self-contained classroom; she receives speech, occupational, and physical therapy. At the 7-year-old well child visit, her mother is worried about Kelly's poor progress in school, and she expresses concern about her daughter's hearing. Her pediatrician observes that Kelly is withdrawn, uses minimal language, and is fearful of the examination. Kelly was born full-term by Cesarean section because of placental abruption. She was in the neonatal intensive care nursery for 2 weeks with metabolic acidosis because of acute tubular necrosis. One day after arriving home, she had a cardiopulmonary arrest followed by emergency open-heart surgery for critical pulmonary hypertension. Her postoperative course was significant for renal failure, extracorporal membrane oxygenation, ventilator dependency, tracheostomy, and gastrostomy. By 3 years of age her medical condition stabilized, and the tracheostomy and gastrostomy tubes were removed. A review of Kelly's previous audiological tests revealed a failed otoacoustic emission test at 5 months. An auditory brain stem response test at 8 months recorded normal hearing in the right ear. At 4 years, behavioral audiometry was attempted but not completed because Kelly cried throughout the session. At 5 years, testing with ear inserts showed normal hearing bilaterally. Because of the concerns raised by Kelly's mother at the pediatric visit, she was referred to audiology for a reevaluation. Testing at this time revealed moderate to profound sensorineural hearing loss in both the ears, which was confirmed on subsequent examinations. Kelly was promptly fitted for hearing aids. Her individual education plan was changed to reflect the diagnosis of hearing impairment, and hearing services were implemented in the classroom. On a recent follow-up visit, Kelly was talkative, engaging, and cheerful.

Cochlear Implant Failure due to Cochlear Nerve Deficiency in a Child with Normal Internal Auditory Canal

The cochlear nerve, which propagates electrical signals to the auditory cortex, must function well for successful cochlear implantation. A narrow internal auditory canal (IAC) increases the risk of hypoplasia or aplasia of cochlear nerve, which may result in the failure of cochlear implantation. Therefore, a functioning cochlear nerve must be verified before deciding to do cochlear implantation by means of behavioral audiometry, electrophysiologic tests, or imaging studies in cases with narrow IAC. On the other hand, a candidate with a normal IAC can be overlooked in investigating the presence of functioning cochlear nerve. Because normal IAC does not always guarantee the presence of normal cochlear nerve, the functioning cochlear nerve should be proved prior to cochlear implantation in those cases. We report on a child, who had normal IAC and cochlea but failed auditory stimulation after cochlear implantation, and who was diagnosed as having cochlear nerve deficiency through retrospective review.

Failure to detect deaf‐blindness in a population of people with intellectual disability

Early identification of deaf-blindness is essential to ensure appropriate management. Previous studies indicate that deaf-blindness is often missed. We aim to discover the extent to which deaf-blindness in people with intellectual disability (ID) is undiagnosed. A survey was made of the 253 residents of an institute offering residential and occupational facilities for people with IDs. Data are included for the 224 individuals who were able to complete both auditory and visual assessments. Otoacoustic emissions were used to screen for hearing impairment; those who did not pass were assessed by behavioural audiometry. Visual acuity was assessed with one of the following: EH-Optotypes, LH-Optotypes, Teller Acuity Cards, Cardiff Acuity Cards or the Stycar Ball Vision Test. Prior to the study hearing impairment had been diagnosed in 12.5% of the 224 subjects, and visual impairment in 17%. Upon completion of the study these figures rose to 46% and 38.4% respectively. Deaf-blindness was diagnosed in 3.6% of the subjects before, and in 21.4% after, the study. Most (87.5%) of the deaf-blind individuals had profound ID. Deaf-blindness is most often not identified either by standard medical screening or by care staff. Individuals with this disability, however, require provision of special kinds of care. Four categories of deaf-blindness are proposed, according to the severity of sensory impairment in each modality. The tests used in this study are non-invasive and are appropriate for individuals with ID and children. Early and periodic screening for visual and hearing impairment in individuals with ID is recommended.

Delayed Recognition of Profound Hearing Loss in a 7-Year-Old Girl With a Neurological Condition

Kelly is a 7-year-old girl with a complex medical history including asthma, mild spastic diplegia, and seizure disorder that is controlled with carbamazepine. She had a significant receptive and expressive language impairment and milder delays in gross and fine motor skills. Kelly is currently repeating first grade in a self-contained classroom; she receives speech, occupational, and physical therapy. At the 7-year-old well child visit, her mother is worried about Kelly's poor progress in school, and she expresses concern about her daughter's hearing. Her pediatrician observes that Kelly is withdrawn, uses minimal language, and is fearful of the examination.Kelly was born full-term by Cesarean section because of placental abruption. She was in the neonatal intensive care nursery for 2 weeks with metabolic acidosis because of acute tubular necrosis. One day after arriving home, she had a cardiopulmonary arrest followed by emergency open-heart surgery for critical pulmonary hypertension. Her postoperative course was significant for renal failure, extracorporal membrane oxygenation, ventilator dependency, tracheostomy, and gastrostomy. By 3 years of age her medical condition stabilized, and the tracheostomy and gastrostomy tubes were removed.A review of Kelly's previous audiological tests revealed a failed otoacoustic emission test at 5 months. An auditory brain stem response test at 8 months recorded normal hearing in the right ear. At 4 years, behavioral audiometry was attempted but not completed because Kelly cried throughout the session. At 5 years, testing with ear inserts showed normal hearing bilaterally.Because of the concerns raised by Kelly's mother at the pediatric visit, she was referred to audiology for a reevaluation. Testing at this time revealed moderate to profound sensorineural hearing loss in both the ears, which was confirmed on subsequent examinations. Kelly was promptly fitted for hearing aids. Her individual education plan was changed to reflect the diagnosis of hearing impairment, and hearing services were implemented in the classroom. On a recent follow-up visit, Kelly was talkative, engaging, and cheerful.